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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Regencrative nodular hyperplasia (RHN) is a rare condition, the diagnosis of which is based upon histological findings. It is seen in Felty's syndrome with portal hypertension (PHT), as was the case in the patient reported here. This was a 72-year-old man, with long standing rheumatoid arthritis, hepatosplenomegaly, a neutrophil leucopaenia and oesophageal varices responsible for recurrent haematemeses. Despite a portocaval anastomosis, the patient died from postoperative acute hepatic failure. Histological study revealed changes in the hepatocytes and the reticulin system typical of RNH without cirrhosis. The relationship between Felty's syndrome and RHN, as well as the mechanism of the hypertension, are discussed in the light of cases from the literature.
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PMID:[Anatomo-clinical study of a case of regenerative nodular hyperplasia of the liver with Felty's syndrome and portal hypertension]. 54 55

Report of 2 siblings, aged 12 1/2 and 9 years, with congenital hepatic fibrosis and polycystic disease of the kidneys. Hepatosplenomegaly had been noted in both children at birth. The younger child had suffered from oliguria aged 2 1/2 years. At diagnosis both children had low platelet counts, one also had leucopenia. The cystic disease of the kidneys was verified by angiography. Coeliacography and splenopartography were diagnostically irrelevant. The diagnosis only became apparent from liver biopsy which was performed during splenectomy. After splenectomy there was an increase of platelets, white blood cells and the clotting factors II, V and X. The three years follow-up showed a constancy of renal impairment and of the minor oesophageal varices observed in the one patient who did not have a spontaneous spleno-renal anastomosis. So far no bleeding has been observed. Porto caval anastomosis was omitted in both children. Pros and cons are being discussed.
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PMID:[Congenital hepatic fibrosis and polycystic disease of the kidneys in two siblings (author's transl)]. 55 82

Histiocytosis X describes a disease characterized by histiocytic infiltration of the reticuloendothelial system, skin, bones, and pituitary gland. The disseminated form frequently occurs in infants and children. Chemotherapy has significantly improved the prognosis in this disorder. Sixty-three per cent of survivors, however, have some residual disability related to fibrosis of tissues previously infiltrated by histiocytes. In instances of liver involvement, healing by fibrosis may result in cirrhosis with portal hypertension and bleeding esophageal varices. Clinical findings include hepatosplenomegaly, jaundice, ascites, hypoalbuminemia, prolonged prothrombin time, and Bromsulphalein retention. Histologic examination of the liver shows a characteristic dense "macronodular" periportal cirrhotic pattern. Three children with portal hypertension and bleeding varices due to healed histiocytosis X were sucessfully managed by portosystemic shunt procedures. Portacaval, mesocaval, and central splenorenal shunts were equally effective in relieving poral hypertension. These children had neither recurrence of bleeding nor evidence of encephalopathy. Two children remain well whereas in one patient a primary hepatoma developed fourteen years posthung and he died of pulmonary metastases. Portosystemic shunt procedures effectively relieve the threat of potentially fatal variceal hemorrhage and improve the opportunity for long-term survival in children with cirrhosis and portal hypertension due to healed histiocytosis X.
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PMID:Portal hypertension in infants and children with histiocytosis X. 108 50

HSS represents a special model of intrahepatic portal hypertension characterized by a presinusoidal portal block and a well-preserved liver parenchyma. Symmers' fibrosis appears in a small but significant proportion of patients with a high worm load. Its pathogenesis is not well established, although experimental and clinical studies point to egg granulomata as the main pathogenetic factor. The eggs carried continuously through the portal circulation produce inflammation and gross amputation of the intrahepatic veins, portal and periportal granulomas, and, eventually, a coarse perilobular fibrosis ("pipe-stem"). Portal hypertension, esophageal varices, and hepatosplenomegaly are the main consequences of these morphologic changes. Gastrointestinal bleeding is the most frequent cause of death. Unlike in cirrhosis, advanced liver failure is not seen except when HSS is associated with liver lesions from other causes such as virus and alcoholism. Helminthiasis treatment is based on chemotherapy with praziquantel or oxamniquine. Bleeding esophageal varices are managed by sclerotherapy or surgical procedures. Splenectomy with gastroesophageal devascularization seems to be the best choice.
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PMID:Hepatosplenic schistosomiasis. Pathophysiology and treatment. 156 71

This study was conducted on 40 patients with schistosomal hepatosplenomegaly and oesophageal varices "O.V.". They were divided into two groups: Group A were suffering from bleeding O.V and group B were non bleeders. Ten Healthy individuals were also included in the study as control group. Plasma fibronectin concentration was estimated in each subject using quantitative single radial immuno-diffusion technique. The results of the study showed that plasma fibronectin concentrations were significantly decreased in both groups of patients, bleeders (354.5 Mg/dl +/- 109.2) and non bleeders (307 +/- 103.5) compared to the control group (514 +/- 57.9). However, there was no significant difference in fibronectin concentration between group A and group B (p greater than 0.05). There was insignificant correlation between plasma fibronectin and some haemostatic estimations (clotting time, bleeding time and platelet count) in the studied groups (p greater than 0.05). Also, there was insignificant difference in fibronectin concentration in patients with (312 Mg +/- 116.4) and without (368.6 +/- 106.9) liver cell failure.
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PMID:Plasma fibronectin concentrations in bleeder and non bleeder patients with hepatosplenic schistosomiasis. 179 63

A 29-year-old male was diagnosed as having essential thrombocythemia (ET) in 1975. From that time, his platelet count gradually increased to more than 2 X 10(6)/microliter until 1979. However, his platelet count gradually decreased to less than 6 X 10(5)/microliter in 1985. Also, in 1982, erythroblasts and immature myeloid cells began to appear in the peripheral blood, and the liver and spleen became palpable in 1985. Bone marrow then revealed osteomyelosclerosis. These findings suggested that ET had transformed to myelofibrosis with myeloid metaplasia. Increased hepatosplenomegaly was accompanied by the appearance of ascites in June, 1988, and an esophageal varix ruptured in December of the same year. The varix was resected and the spleen was removed. After the operation, ascites did not recur and his condition became stable. Portal hypertension in this patient was considered to be due mainly to increased blood flow from the enlarged spleen.
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PMID:[Essential thrombocythemia transformed to myelofibrosis with myeloid metaplasia after seven years]. 204 Nov 65

The clinical, angiographic, and pathologic features of hepatoportal sclerosis in five children are presented. Hepatosplenomegaly with preservation of liver function and esophageal varices were demonstrated. Histologically, portal alterations were consistent and prominent and included subintimal sclerosis of the intrahepatic venous branches and telangiectases. Angiographic patterns were variable, but all exhibited intrahepatic portal venous obstruction. In three patients, there was an association with extrahepatic portal vein obstruction at different sites. Our data suggest that intrahepatic portal vein thrombosis could be the primary disorder in hepatoportal sclerosis, which may vary in extent and site, involving the intrahepatic branches of the portal vein with or without the involvement of the portal trunk. Combined clinical, angiographic, and pathologic data can contribute to a better understanding of hepatoportal sclerosis in children.
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PMID:Hepatoportal sclerosis in childhood. 242 92

Autoimmune chronic active hepatitis (ACAH) is an infrequent modality of chronic hepatitis (CH) with clinical and laboratory findings of an autoimmune disorder. Clinical and pathological findings of 7 cases are presented; all were females with ages between 7 and 24 years. Main symptoms and signs were weight loss, malaise, arthralgias, fever, menstrual disturbances, hepatosplenomegaly, jaundice ascites and esophageal varices. Aminotransferases were elevated in all cases, and bilirubin, alkaline phosphatase and gammaglobulins were found to be raised in six. Antinuclear antibodies were positive in 5 cases, smooth muscle antibodies in 3, and antimitochondrial antibodies were detected in one. Morphological changes were those of chronic active hepatitis with variable degrees of fibrosis. Plasma cells were conspicuous. All patients received steroid treatment (Prednisone). It is concluded that the diagnosis of ACAH can be based on clinical and immunological criteria provided other causes of CH such as viral hepatitis, are ruled out.
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PMID:[Autoimmune chronic hepatitis: clinico-pathologic spectrum in 7 cases]. 251 21

A radiological diagnosis of gastric volvulus (GV) was made in 11 of 576 consecutive upper gastrointestinal series at the University of Benin Teaching Hospital, Nigeria, over a two-year period. The clinical symptoms were thoracico-abdominal in three and abdominal in eight; these cases were evaluated as acute in three, acute upon chronic in two, and chronic in six. There was a significant delay in the diagnosis in all cases (except a neonate in the series), and no case was diagnosed on clinical grounds alone.THE PREDISPOSING FACTORS (EXCEPT THE CLINICAL MISDIAGNOSIS OF THE NEONATE) IN SIX OF THE SEVEN CASES THAT CAME TO SURGERY WERE: diaphragmatic hernia and perigastritis (left lung abscess, thoracic empyema), arteriomesenteric compression of the duodenum in pregnancy (peptic ulcer), splenomegaly (hepatosplenomegaly, ascites, esophageal varices), previous gastrojejunostomy (stomal ulcer, left subphrenic abscess) and two cases of intestinal malrotation with mesenteric abnormalities (small bowel obstruction in one and duodenal atresia in the other). In one idiopathic case, gastric outlet obstruction was clinically suspected prior to surgery. Thus, the putative rarity of GV in black Africans is not supported by this experience.Gastric volvulus is a clinico-radiologic entity that may present with a confusing thoracico-abdominal symptom complex. A greater awareness of the radiologic features is quintessential to an expeditious and usually successful surgical management that will avoid potentially serious complications. Negative surgical findings do not exclude GV as the underlying cause of acute abdomens necessitating emergency laparotomies.
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PMID:Volvulus of the stomach: an African series and a review. 356 Feb 44

Peak activity over the spleen as a percentage of peak activity over the liver was measured in 265 (99m)Technetium sulphur colloid liver scintiscans. The value exceeded 70% in 50 cases. In 32 of these cirrhosis was present; the other 18 scans were from patients with a wide variety of conditions, including secondary deposits, hepatitis, and diseases involving the reticuloendothelial system. A measure of the total activity in the spleen was derived from the peak activity and the length of the spleen. In cirrhosis this was closely related to the finding of oesophageal varices thus showing the importance of a collateral circulation (which allows colloid to bypass the liver) in the increased uptake of colloid by the spleen. In eight patients with hepatosplenomegaly due to blood dyscrasia or disease involving the reticuloendothelial system, total activities in the liver and spleen were estimated from the anteroposterior colour dot scan, and both liver and spleen blood flow were measured by methods independent of reticuloendothelial cell function. The results showed that the main factor causing increased uptake of colloid by the spleen in these diseases was an increased blood flow in the spleen relative to that in the liver.
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PMID:Significance of increased "splenic uptake" on liver scintiscanning. 538 28


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