Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital hepatic fibrosis (CHF) is a recognized cause of portal hypertension with oesophageal varices, gastro-intestinal haemorrhage and cholangitis in children without significant impairment of hepatic or renal function. This report describes the varied clinical presentation of CHF as seen at King Faisal Specialist Hospital and Research Centre (KFSH & RC) and emphasizes the clinical patterns that should enable a pediatrician to consider the diagnosis. Fourteen children with CHF were diagnosed between 1981 and 1988. The age at presentation ranged from 1.8-14 years (mean: 7.5 years); clinical manifestations at diagnosis were splenomegaly (12), hepatomegaly (11), failure to thrive (10), marked abdominal distention (4), and fever (4). Liver function tests were normal except for high alkaline phosphatase. Eight patients had polycystic kidneys confirmed on ultrasound examination. Upper gastro-intestinal endoscopy showed oesophageal varices of variable severity in all eight patients examined. Splenoportography revealed splenic vein occlusion in one patient. One patient died within days of admission with convulsions, coma, and aspiration pneumonia. One patient was lost to follow-up. The remaining 12 patients are all alive and receive regular follow-up. Two patients required splenorenal shunt. In view of the prevalence of consanguinity in Saudi Arabia, the diagnosis of CHF should be considered in children with hepatomegaly despite normal liver function tests, and particularly in those with renal abnormalities and/or evidence of portal hypertension.
 ...
PMID:Congenital hepatic fibrosis in Saudi Arabia. 178 58

Portal hypertension is a common pathology in childhood and one of its most common causes is cavernomatosis of the portal vein. This obstruction causes hemodynamic changes which lead to splenomegaly and collateral circulation. Esophageal varices are one of the most important sequelae, which endanger the patient's life because of a bleeding tendency. Ecosonography helps to detect the thickening of the lesser omentum vis a vis the aortic diameter, caused by the collateral circulation. We studied 15 children presenting with portal hypertension resulting from portal vein cavernomatosis; we performed an upper GI endoscopy and abdominal ecosonography. The endoscopy revealed grade II esophageal varices in 20% of cases, the remaining 80% had grade III and grade IV. Ecosonography revealed an increased lesser omentum/aorta ratio in children with portal hypertension, compared to controls (p < 0.001). Our results suggest that the lesser omentum/aorta ratio has diagnostic value in pediatric portal hypertension.
 ...
PMID:[Predictive value of ultrasonography in portal hypertension]. 184 29

Patients with portal hypertension without a demonstrable cause have been reported in the literature under several different terms, such as tropical splenomegaly, phlebosclerosis, obliterative portal venopathy of the liver, hepatoportal sclerosis, noncirrhotic portal fibrosis and idiopathic portal hypertension (IPH). Such patients have been described worldwide, with a greater frequency in India and Japan. The etiology of IPH is still unknown, although some of the theories that have been proposed are: exposure to toxic substances or drugs, relationship with the hepatitis-B virus, immunologic abnormalities, systemic or intra-abdominal infections and clotting abnormalities. The main histopathologic findings are periportal fibrosis, obliteration of small portal veins and sclerosis of the interhepatic portal system. Although these abnormalities could be secondary to portal hypertension, it has been proposed that the vascular changes are the primary event that leads to portal hypertension. The site of increased resistance in IPH is found at the presinusoidal level with some component at the sinusoidal and postsinusoidal level. The main symptoms and signs in IPH are upper gastrointestinal tract bleeding secondary to esophago-gastric varices, symptoms related to anemia, and splenomegaly. The long-term prognosis for patients with IPH is better than for cirrhotic patients, with a 77% survival at ten years. Variceal bleeding is the main cause of death, and some treatment to prevent bleeding or its recurrence is warranted. Although no comparative trial has been performed in IPH patients, the surgical management could be the first choice for elective treatment in these patient without liver failure, because of the high re-bleeding rates with chronic sclerotherapy. Pharmacologic management could be considered for prophylactic treatment of these patients.
 ...
PMID:[Idiopathic portal hypertension]. 186 3

The authors report the cases of five children in whom kala-azar was undiagnosed at first instance. In these cases, the diagnosis was misled because of incomplete features (lack of fever, splenomegaly or hypergammaglobulinemia) an associated disease (hydatic cyst of the liver) or a complication dominating the clinical pattern (septicemia, staphylococcus respiratory infection). In one case, the patient was explored in order to diagnose portal hypertension.
 ...
PMID:[Misleading forms of visceral leishmaniasis in children. Apropos of 5 cases]. 194 39

Non-cirrhotic portal hypertension (NCPH) is a rare cause of bleeding oesophageal varices. The prognosis for patients with NCPH is generally better than that of patients suffering from cirrhotic portal hypertension. Gastrointestinal bleeding or asymptomatic splenomegaly is the usual clinical presentation. If surgery becomes necessary splenectomy alone is therapeutically insufficient. Definitive reduction of portal pressure by complete or incomplete shunts provide the lowest rate of recurrent bleeding and an excellent long-term prognosis. Exact diagnosis of the aetiology of NCPH is of great importance in the correct choice of operative procedure.
 ...
PMID:[Hemorrhage from esophageal varices in non-cirrhotic portal hypertension]. 198 81

A 61-year-old man presented with ascites in the course of agnogenic myeloid metaplasia (AMM). Ascitic fluid was exudative and contained mature and immature leukocytes, erythroid cells, and megakaryocytes as observed on a bone marrow smear. Peritoneal biopsy showed myeloid metaplasia, and liver biopsy revealed intrasinusoidal myeloid metaplasia and peliosis. Ascites cleared after abdominal radiotherapy but treatment resulted in transient aplasia. Subsequently, portal hypertension was demonstrated by hepatic transjugular catheterization. Complications of splenomegaly led to splenectomy and splenorenal shunt followed by fatal acute hepatitis and septic shock. A review of the literature and an analysis of mechanisms of ascites occurring in AMM, especially peritoneal implants of myeloid tissue and occurrence of peliosis in myeloproliferative disorders, are presented.
 ...
PMID:Ascites revealing peritoneal and hepatic extramedullary hematopoiesis with peliosis in agnogenic myeloid metaplasia: case report and review of the literature. 198 77

A 40-year-old man with rheumatoid arthritis, splenomegaly and mild thrombocytopenia presented with gross ascites and a history of excess alcohol consumption. Oesophageal varices were documented endoscopically. Alcoholic cirrhosis was suspected and laparoscopy revealed a macronodular liver surface. Liver biopsy disclosed subtle microscopic structural variations which together with the laparoscopic findings were consistent with the diagnosis of nodular regenerative hyperplasia. The importance in diagnosis of macroscopic appearance combined with histological findings is emphasised. Clinically significant portal hypertension may be present at a histologically early stage of this condition.
 ...
PMID:Nodular regenerative hyperplasia of the liver: the importance of combined macroscopic and microscopic findings. 200 57

In this study, performed to assess the effect of auxiliary heterotopic liver transplantation on portal hypertension and hypersplenism, eight patients with chronic liver disease who underwent the procedure and had functioning grafts for at least 6 months were analyzed. The transplantation resulted in (a) normalization of platelet and leukocyte counts, (b) reduction of splenomegaly by 20% +/- 3% (P less than 0.02), (c) disappearance of ascites, and (d) diminution of esophageal varices in all patients. Intraoperatively, the mean portacaval pressure gradient decreased with 54% +/- 7% after recirculation of the graft (P less than 0.05). In conclusion, a functioning auxiliary heterotopic liver graft decompresses portal hypertension and reverses hypersplenism.
 ...
PMID:Does auxiliary heterotopic liver transplantation reverse hypersplenism and portal hypertension? 835 66

Sixteen cases of nodular regenerative hyperplasia of the liver in children are presented. The patients, 10 girls and 6 boys, were between the ages of 7 months and 13 years, with a median of 6 years. Clinically, nine children presented with hepatomegaly or splenomegaly, with and without signs of portal hypertension. A history of anticonvulsant drug therapy was obtained in four patients. Associated conditions in the remaining three cases were Donohue's syndrome, disseminated intravascular coagulation, and angiomyolipoma of the kidney. In five patients a clinical diagnosis of primary intra-abdominal tumor was made. Follow-up showed that six patients died of causes unrelated to the nodular hyperplasia. Two patients were asymptomatic when last seen 5 and 18 years after the initial diagnosis of nodular hyperplasia. Both patients underwent shunt surgery. No follow-up was available for eight patients. The importance of recognizing this entity in the pediatric age group, as well as its histopathologic differential diagnosis, is stressed.
 ...
PMID:Nodular regenerative hyperplasia of the liver in children. 203 39

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.
 ...
PMID:[Essential thrombocythemia transformed to myelofibrosis with myeloid metaplasia after seven years]. 204 Nov 65


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>