Immediate graft histological assay, post pig's liver transplantation

PURPOSE: To describe the vascular and tissue histopathological changes in seven sequential experimental liver transplantations in pigs. METHODS: Fourteen female pigs, Sus domesticus species, with body mass between 5 and 8 kg were utilized. After the end of all anastomoses of the graft implantation in the receptor, the animal was monitored for 30 minutes, and at its end one of the biopsies was collected for histological analysis. The histological criteria utilized were: lytic hepatocyte necrosis, density of septal and portal inflammatory infiltrated, sinusoidal congestion and hemorrhage. The analysis was performed separately for the portal region in zone 1, 2 and 3. RESULTS: Among the structural changes undergone by the graft, those with greater frequency and intensity were vascular congestion and steatosis, which stood out in transplantations 5, 6 and 7. CONCLUSIONS: The technique demonstrated vascular alterations represented by vasocongestion, edema and minimum inflammatory reaction. In relation to the parenchyma, was observed macrovacuolar pan-acinar steatosis, focal lytic and occasional hemorrhages, beyond the accumulation of hemosiderin in Kuppfer's cells.

Cavernous transformation of portal vein - a rare cause of massive hepatomegaly

There are very few reported cases of Cavernous Transformation of Portal Vein [CTPV] in adults. We present a case of 79 years old male who was found to have this complication due to portal vein thrombosis [PVT]. A 79 year old male with background history of JAK2 positive Myeloproliferative disorder [MPD] was referred with abnormal liver function tests. Patient was clinically well and asymptomatic. During initial workup for his abnormal LFTs, patient was noted to have enlarged caudate lobe of liver. Further abdominal imaging studies showed massively enlarged caudate lobe of liver with Cavernous Transformation of Portal Vein [CTPV], a very rare complication of portal venous thrombosis. Cavernous transformation of portal vein is a very rare cause of enlarged caudate lobe of liver. The management of CTPV is mainly symptomatic. Most of the patients are asymptomatic at presentation. Complications mostly occur due to portal hypertension which can be life threatening. There is no consensus on the management of Cavernous Transformation of portal vein itself. Patients with cavernous transformation of portal vein should be kept under regular follow up

Immunohistochemical studies of stellate cells in experimental cholestasis in newborn and adult rats

BACKGROUND AND AIMS: Although there is much known about liver diseases, some aspects remain unclear, such as the nature of the differences between the diseases observed in newborn infants and those in adults. For example, how do newborns respond to duct epithelial cell injury? Do the stellate cells in newborns respond similarly to those in adults during biliary obstruction? METHODS: Ninety newborn Wistar rats aged six days, weighing 8.0 - 13.9 g each, and 90 adult rats weighing 199.7 - 357.0 g each, were submitted to bile duct ligation. After surgery, they were randomly divided and sacrificed on the 3rd, 5th, 7th, 14th, 21st or 28th day post-bile duct ligation. Hepatic biopsies were obtained and immunohistochemical semi-quantification of desmin and á-SMA expression was performed in hepatic stellate cells and in myofibroblasts in the portal space, and between the portal space and the liver lobule. RESULTS: Desmin expression in the myofibroblast cells post-bile duct ligation was higher in young rats, reaching its peak level in a shorter time when compared to the adult animals. The differences between the groups for á-SMA expression were less significant than for desmin. CONCLUSIONS: These findings indicate that there is an increase in the number of collagen-producing myofibroblast cells in young animals, suggesting that there is more intense fibrosis in this population. This finding may explain why young animals with bile duct obstruction experience more intense portal fibrosis that is similar to the pathology observed in the livers of newborns with biliary atresia.

Dorfman-Chanarin syndrome.

A four-year-old girl was brought to the dermatology outpatient department with scaling all over the body since birth. She had history of episodic vomiting and abdominal distension. A dermatological diagnosis of lamellar ichthyosis was made. Abdominal examination revealed a nontender hepatomegaly, fatty liver on ultrasonography and deranged liver function tests. Peripheral blood smear showed lipid vacuoles in the granulocytes consistent with Jordans' anomaly. Similar lipid vacuoles were seen in the basal layer in skin biopsy. An inflammatory infiltrate, moderate fibrosis in the portal tract and diffuse severe fatty change in hepatocytes were seen in liver biopsy. The patient was diagnosed as a case of Dorfman-Chanarin syndrome.

Remodeling of hepatic vascular changes after specific chemotherapy of schistosomal periportal fibrosis

Hepatosplenic schistosomiasis was the first human disease in which the possibility of extensive long standing hepatic fibrosis being degraded and removed has been demonstrated. When such changes occurred, the main signs of portal hypertension (splenomegaly, esophageal varices) progressively disappeared, implying that a profound vascular remodeling was concomitantly occurring. Hepatic vascular alterations associated with advanced schistosomiasis have already been investigated. Obstruction of the intrahepatic portal vein branches, plus marked angiogenesis and compensatory hyperplasia and hypertrophy of the arterial tree are the main changes present. However, there are no data revealing how these vascular changes behave during the process of fibrosis regression. Here the mouse model of pipestem fibrosis was used in an investigation about these vascular alterations during the course of the infection, and also after treatment and cure of the disease. Animals representing the two polar hepatic forms of the infection were included: (1) "isolated granulomas" characterized by isolated periovular granulomas sparsely distributed throughout the hepatica parenchyma; and (2) 'pipestem fibrosis' with periovular granulomas and fibrosis being concentrated within portal spaces, before and after treatment, were studied by means of histological and vascular injection-corrosion techniques. Instances of widespread portal vein obstruction of several types were commonly found in the livers of the untreated animals. These obstructive lesions were soon repaired, and completely disappeared four months following specific treatment of schistosomiasis. Treatment was accomplished by the simultaneous administration of praziquantel and oxamniquine. The most impressive results were revealed by the technique of injection of colored masses into the portal system, followed by corrosion in strong acid. The vascular lesions of non-treated pipestem fibrosis were represented...

[Biliary abnormalities associated to portal cavernoma: about 17 cases]

Portal biliopathy is a newly introduced term, describing the changes observed in the biliary ducts secondary to extrinsic compression by the portal cavernoma. It's a rare condition that is usually not diagnosed. To explore the principles of diagnosis and treatment of portal biliopathy. Seventeen patients with extrahepatic portal vein obstruction were reviewed prospectively. Symptomatic biliary obstruction was found in 82%. Endoscopic retrograde cholangiography revealed abnormality of the bile duct wall in all cases, with stricture in 11 patients. Endoscopic treatment was necessary in 10 patients. Whether portosystemic shunting was done only in 2 cases. Portal biliopathy is frequent and must be diagnosed early

On the presence of hepatic stellate cells in portal spaces

Previous studies in mice with hypervitaminosis A have demonstrated that fat-storing cells (hepatic stellate cells-HSCs) participate in schistosomal granuloma fibrogenesis. The origin of such cells in portal areas, away from the Disse spaces, was herein investigated. HSCs were identified in frozen sections of the liver by means of Sudan III staining. They appeared as red-stained cells disposed along the sinusoids of normal mice, but were never found within portal spaces. However, in the chronically inflamed portal spaces of Capillaria hepatica-infected mice, Sudan III-positive cells were frequently present among leukocytes and fibroblast-like cells. Thus, there are no resident HSCs in portal spaces, but their presence there in chronic inflammatory processes indicates that they are able to migrate from peri-sinusoidal areas in order to reach the portal areas.

Non-cirrhotic portal fibrosis in children.

BACKGROUND AND OBJECTIVES: Non-cirrhotic portal fibrosis (NCPF) is an infrequent cause of portal hypertension in children. We report 11 children with NCPF, from among 338 with portal hypertension, seen over 6.5 years. METHODS: The diagnosis was based on patent splenoportal axis on ultrasonography and/or splenoportal venography and liver biopsy showing no evidence of cirrhosis or other diagnosis, in children with portal hypertension. Those with variceal bleed were managed with endoscopic sclerotherapy and/or shunt surgery. RESULTS: The median age was 11 years (range 5 to 14), and 8 were boys. Presentation was with variceal bleed in 6, lump in left upper abdomen in 5 (though all children had splenomegaly) and esophageal varices on endoscopy. The median spleen enlargement was 8.5 cm; 8 also had hepatomegaly. Hypersplenism was present in 7, and two had developed ascites after bleed. Of 6 children presenting with bleed, variceal obliteration was achieved on sclerotherapy (average 5.6 sessions) in 4 while two underwent shunt surgery for associated hypersplenism. After median follow up of 57.5 months (range 12-78) all are alive and well. CONCLUSION: NCPF is an uncommon cause of portal hypertension in Indian children. Presentation with variceal bleed is less common than in adults; sclerotherapy is effective.

Thrombosis of the portal system following splenectomy

Venous thrombosis of the portal system following splenectomy is uncommon, with an incidence of 0,2 to 6%. The clinical presentation varies greatly and the diagnosis is difficult and must be confirmed by doppler ultrasonography. The authors report the study of 2 observations. Diagnosis, therapeutic and prophylactic aspects of this complication of splenectomy are discussed

Esclerosis hepato-portal: 10 anos de experiencia el el Instituto Nacional de Pediatria / Hepatoportal sclerosis. 10 years of experience in the National Institute of Pediatrics

Objetive: To assess the frecuency and clinical picture of Hepatoportal Sclerosis in a population of Mexican children of the Instituto Nacional de Pediatría, México City. Background: Hepatoportal Sclerosis is a disease of unknown etiology. It's diagnosis is difficut. The main clinical presentation is splenomegaly with or without hematemesis (portal hypertension). Splenoportography and liver histology study are the best procedures for diagnosis and must be performed by experts. Methods: We studied 7/106 children with portal hypertension during a period of 10 years, who were seen at the Instituto Nacional de Pediatría, México city. Inclusion criteria were specifical findings of splenoportography and histologic changes in liver biopsy. Results: We found 7/106 children. The main clinical manifestation were splenomegaly and hematemesis. We did not find any previous history of contact with arsenisc, vinyl chloride or copper sulfate. In 6/7 children a porto-systemic shunt was performed. Only one received propranolol and sclerotheraphy. At the time of this report all children have shown a good clinical course.

Correlation between the degree of periportal thickening, portal blood flow and splenic volume

Schistosomiasis leads to presinusoidal hepatic fibrosis which determines the prognosis of the disease and has variable sequences on the portal haemodynamics. In order to evaluate the relation between the degree of hepatic fibrosis, portal hypertension and splenic volume by means of ultrasound and Duplex Doppler, 35 schistosomal patients [25 males and 10 females with a mean age of 37 years] showing various degrees of periportal fibrosis were studied. The grade of periportal fibrosis, portal blood flow, portal vein diameter and splenic volume were determined by means of ultrasound and Duplex Doppler. The portal vein diameter showed a mean of 10.8 mm, portal blood flow showed a mean of 877 cm 3 and the splenic volume had a mean of 149 cc. There was a positive significant linear correlation between the portal vein diameter and the grade of fibrosis the portal blood flow and the grade of fibrosis and the splenic volume and the grade of fibrosis. In conclusion, hepatic periportal fibrosis affects the portal haemodynamics. It correlates positively with the portal blood flow and the splenic volume. Furthermore ultrasound and Duplex Doppler are simple, non invasive techniques for evaluation of haemodynamic changes in schistosomal periportal fibrosis

Avaliaçäo do método ultrassonográfico no diagnóstico da hipertensäo portal: análise de 30 casos / Evaluation of ultrasonographic method in the diagnosis of portal hypertension: analysis of 30 cases

Os autores estudaram 30 pacientes com hepatopatias crônicas (24 cirróticos, 04 esquistossomóticos hepatoesplênicos, 01 com cirrose e esquistossomose e 01 com hipertensäo portal idiopática) através de ultrassonografia abdominal, com o objetivo de verificar a presença de hipertensäo portal e analisar seus diversos aspectos de forma rápida e näo invasiva. Em cada um dos pacientes foram investigadas as alteraçöes morfológicas e parenquimatosas do fígado e baço, visualizando-se concomitantemente a vesícula, vias biliares e pâncreas, com ênfase ao calibre e trajeto dos principais vasos componentes do sistema portal e da circulaçäo colateral adquirida