Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A rare chronic course of Budd-Chiari syndrome associated with thrombosis of the portal vein was observed in a 30-year-old male patient suffering from postmyocarditic cardiosclerosis. At the age of 24 the patient had infectious allergic myocarditis, was hospitalized and rehospitalized for circulatory insufficiency. Upon 3 years since the disease onset the patient was admitted to a hematological department for progressive enlargement of the spleen. The diagnosis on discharge was idiopathic myelofibrosis with portal hypertension. The treatment included prednisolone, blood transfusions, myelosan. In 1987 the patient presented with enlarged liver and spleen, ascites, gastric and esophageal varicosis, augmenting hepatic insufficiency clinically evaluated as hepatic cirrhosis. Postmortem examination revealed macrofocal cardiosclerosis, splenomegaly, ascites, portal varicosis, enlarged nutmeg liver with smooth surface. Microscopically there was phlebosclerosis and phlebothrombosis varying in duration and involving predominantly medial branches of the hepatic and portal veins, liver fibrosis. The findings provided evidence for the final diagnosis of Budd-Chiari syndrome running an uncommon chronic course.
 ...
PMID:[The chronic form of the Budd-Chiari syndrome]. 297 4

We report an unusual case of lambda light chain deposits without overt plasma cell dyscrasia. The clinical presentation was hepatomegaly without biochemical sign of renal involvement. Portal hypertension, spontaneous rupture of the spleen and fracture of the 12th thoracic vertebra occurred during the course of the disease. Ultrastructural studies showed that lambda light chain deposits were associated with amyloid-like deposits. This case suggests that light chain deposition disease and amyloidosis could be two expressions of the same disease.
 ...
PMID:Light chain deposition disease presenting with hepatomegaly: an association with amyloid-like fibrils. 315 78

A cross-sectional study was made of the morbidity due to Schistosoma mansoni infection in the Rusizi plain, Burundi. An evenly distributed 5% population sample (n = 6203) was examined; each subject was submitted to a standardized medical history and abdominal palpation. The prevalence of infection was 33% and most infections were light. Diarrhoea was complained of by 26% of those infected and 21% of those not infected; "bloody diarrhoea" by 13% and 4%, respectively. The association with schistosomiasis was significant in all age groups. "Abdominal pain" was a very common complaint, "tiredness" an infrequent one; neither was associated with the infection. Left lobe hepatomegaly was found in 26% of those infected, and in 10% of those not infected; right lobe hepatomegaly in 7% and 5%, and splenomegaly in 30% and 24%, respectively. The frequency of organomegaly and its association with schistosomiasis was maximal in children, decreased in adolescents and young adults, and increased again in older adults; its intensity was generally mild. Ascites or histories of haematemesis were not recorded, though several cases of decompensated portal hypertension due to schistosomiasis have been documented at the central hospital of Bujumbura. The relation of morbidity to intensity of infection was limited to a correlation between hepatomegaly and egg load in those over 40 years old. It is concluded that, in this situation, selective mass treatment is a better strategy than targeted or selected group chemotherapy.
 ...
PMID:The morbidity of schistosomiasis mansoni in the Rusizi Plain (Burundi). 315 15

Late symptoms of infantile cystinosis were evaluated in 19 patients aged 15-26 years who had a high graft survival following kidney transplantation. The end-stage cystinotic kidney was responsible for renal hypertension in 5 patients following grafts. Photophobia did not increase in relation to age, but 3 patients became blind and 1 lost the sight in one eye at 25 years of age. Two patients developed clinical symptoms of hypothyroidism, and 15 other patients had a compensated hypothyroidism. Four patients developed permanent insulin-dependent diabetes and 2 developed transient insulin-dependent diabetes after transplantation. The oral glucose tolerance test was abnormal in 11 of 14 patients on low-dose prednisone. Liver enlargement was noted in 10 cases, but only 3 patients developed clinical symptoms of portal hypertension. Symptoms of hypersplenism were observed in 6 cases leading to splenectomy. Repeat gross epistaxis was observed in 7 of the patients when on dialysis and persisted after transplantation in 1 patient, who died from nasal bleeding. A particular encephalopathy developed in 2 patients at the ages of 17 and 24, characterized by speech difficulties, pyramidal symptoms and cranial nerve deficit; one died at the age of 21. The mean adult height of these patients was 136.5 cm in males and 124 cm in females, and their psychosocial adjustment was related to the extra-renal complications of cystinosis rather than to the renal status.
 ...
PMID:Late symptoms in infantile cystinosis. 315 26

Fourteen patients had mesenteric, portal, or splenic venous thrombosis that was diagnosed primarily by contrast-enhanced CT. The group included five patients with coagulopathy, three with pancreatic carcinoma, two with cirrhosis and portal hypertension, one with pancreatitis, and one with hepatocellular carcinoma. In two patients, no etiology was determined. In all cases, CT easily identified low-density venous thrombosis, which frequently involved more than one vein. In four patients, all three splanchnic veins were involved; five patients had occlusion of two veins. In five patients, only one vein was involved. Additional CT findings included ascites, collateral veins, hepatomegaly, and splenomegaly. No venous wall enhancement was found. CT also was helpful in defining the cause of thrombosis in six of 14 patients. Mesenteric edema and/or bowel wall thickening was not identified. None of the patients had classic clinical evidence of splanchnic venous occlusion, and none died primarily of that disease. The major morbidity suffered by these patients stemmed from complications of splanchnic venous occlusion, and nine patients ultimately required sclerotherapy, splenectomy, and portal decompression. We conclude that CT is useful in the diagnosis of splanchnic venous thrombosis. Our experience suggests that mesenteric, splenic, and/or portal venous thrombosis may occur more commonly than has been previously thought and that the disease in many cases is not life threatening.
 ...
PMID:Thrombosis of the splanchnic veins: CT diagnosis. 325 38

A 31-yr-old man presented with the Budd-Chiari syndrome. A liver biopsy specimen demonstrated noncaseating centrilobular granulomas and an idiopathic necrotizing granulomatous venulitis involving hepatic vein radicles with superimposed thrombotic occlusion. High-dose prednisone therapy produced dramatic clinical improvement with resolution of the hepatomegaly, ascites, and portal hypertension and normalization of the hepatic venous angiogram. A repeat liver biopsy specimen showed resolution of the venulitis and disappearance of the hepatic vein thrombosis. Idiopathic granulomatous venulitis that is reversible with prednisone therapy should be considered in the differential diagnosis of the Budd-Chiari syndrome.
 ...
PMID:Response to steroids in Budd-Chiari syndrome caused by idiopathic granulomatous venulitis. 333 22

Three hundred and sixteen patients with cystic fibrosis were seen at the Brompton Hospital during 1965-83; 178 (56.3%) of them were male and 136 female, and their ages ranged from 12 to 51 years. Most patients presented in infancy with respiratory symptoms and malabsorption, but 19 (6%) were diagnosed in adult life, three in their 30s. Pulmonary disease was almost universal (99.7%), being responsible for 97% of all deaths and three quarters of hospital admissions. All patients had developed a productive cough by the age of 21 and over half before the age of 5. Many complained of wheezing, but reversible airflow obstruction was present in only 40% of those tested. Minor haemoptysis was very common (62%), but major episodes less so (10%). Pneumothorax was seen in 61 cases (19%), and was often recurrent. Some irreversible airflow obstruction was present in all patients with pulmonary disease. Two patients have been followed for over 20 years without showing appreciable decline in lung function. Thirty five patients (11%) had no symptoms of malabsorption. Acute meconium ileus equivalent was seen in 16% and a chronic partial obstruction with episodic symptoms in a further 19%. Diabetes mellitus developed in 36 patients, 13 of whom were insulin dependent. Hepatomegaly was common (29%), often occurring without abnormal results in biochemical tests of liver function; only 1% of patients developed portal hypertension with varices and ascites. Skin reactions to at least one common allergen, including Aspergillus fumigatus, were positive in 70%, but very few patients suffered from hay fever or eczema. One hundred and twenty one patients have died, 97% from infection or other pulmonary complications, and 195 were alive in December 1983 (mean age 23 years). Seventy eight per cent of patients were in full time education or full or part time employment, or were housewives, and only 41 were unemployed for reasons for health. Many patients are married and 10 women have borne children. Most patients were admitted to hospital only three or four times during the period of follow up and 50 individuals (16%) have never been in hospital at all. The improvement in prognosis and quality of life for adults with cystic fibrosis should encourage a positive attitude in those who care for them.
 ...
PMID:Cystic fibrosis in adolescents and adults. 343 96

The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
 ...
PMID:Paediatric liver disorders in Singapore. 346 38

The clinicopathologic features and natural history of primary sclerosing cholangitis were reviewed in 53 patients followed at the Yale Liver Center during the past 30 yr. At presentation, the mean age of patients was 46 yr, and the male to female ratio was 1.4:1. Biliary sclerosis was limited to the intrahepatic ductal system in 21% of the patients. Fifty-three percent of the patients had mild disease without portal hypertension at presentation, and 25% had no symptoms attributable to their liver disease. Long-term follow-up was available for 42 patients and averaged 56 mo. Over this period, 16 patients remained mildly symptomatic, and 11 were asymptomatic. Survival was calculated by a Kaplan-Meier life-table analysis and demonstrated that 75% of the patients were alive 9 yr after the diagnosis of primary sclerosing cholangitis. A multivariate analysis of clinical features revealed that hepatomegaly and a serum bilirubin level greater than 1.5 mg/dl at the onset of disease were independent discriminators of a poor prognosis. Patients referred to this university medical center displayed different clinical characteristics than previously reported in primary sclerosing cholangitis. A higher percentage were older, female, and asymptomatic, and more had disease limited to the intrahepatic ductal system. Survival was also considerably improved in this group of patients and suggests that the long-term prognosis for patients with primary sclerosing cholangitis may be considerably better than previously believed.
 ...
PMID:Improved survival with primary sclerosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients. 356 62

Nodular regenerative hyperplasia of the liver developed in five patients who had the toxic oil syndrome caused by ingestion of adulterated cooking oil. This hepatic complication was detected from 19 to 37 months (mean-2.5 years) after the onset of the toxic oil syndrome. Nodular regenerative hyperplasia was asymptomatic, although all patients had persistently abnormal liver function. Hepatomegaly was present in four cases, mild jaundice in three and signs of portal hypertension in two. Pathogenesis of nodular regenerative hyperplasia in toxic oil syndrome is unknown, but probably microcirculatory disturbances within the liver might have played a role. Fibrosis in Zone 3, sinusoidal dilatation and occasionally intralobular hemorrhage were seen in three cases; in one of them, characteristic lesions of veno-occlusive disease were also present. In another case, endarteritis changes of hepatic arterioles were evident.
 ...
PMID:Nodular regenerative hyperplasia of the liver associated with the toxic oil syndrome: report of five cases. 373 2


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