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

We describe retrospectively the experience with 44 cases of AIDS from January 1987 to October 1991 at the Instituto Nacional de Pediatria, a tertiary care children hospital in Mexico City. All patients with 2 ELISA and a positive Western Blot test were included. Thirty three patients were infected perinatally (75%) and 11 through blood transfusion (25%). Fourty one patients belonged to the P2 classification of the Centers for Disease Control. Chronic diarrhea (77%), lymphadenopathy (75%), hepatomegaly/splenomegaly (70%) and oral candidiasis (61%) were the most common clinical findings. Twenty patients died (45.4%). No statistical relation were found between survival rate and the way of transmission and age at onset. Autopsy was performed in 14 patients and revealed a sharp decrease of lymphoid tissue at all levels with severe thymic atrophy.
Bol Med Hosp Infant Mex 1992 Sep
PMID:[AIDS in children. Experience at the National Institute of Pediatrics]. 138 80

Over a 6-year period, in 1425 adult computed tomographic studies, radiological evidence of fatty infiltration of the liver (FIL) was found in 138 patients (9.7%). Patients with FIL had a mean age +/- SD of 45.9 +/- 15.7 years and 57% were males; the majority were Saudis (73%). Most patients (95%) had one or more underlying aetiological causes. Haematological and non-haematological malignancies with or without liver involvement were the most frequently encountered aetiological factors (66% of patients). FIL contributed to hepatomegaly or was associated with abnormality in one or more of the liver function tests in 30% and 39% of patients, respectively. Assessment of the various radiological patterns showed diffuse fatty changes in 68% of patients and solitary or multiple focal changes in 9% and 22%, respectively. 13 patients (9%) showed sparing of the caudate lobe within a diffuse fatty process. Patients with diffuse FIL had significantly higher values for alkaline phosphatase (p = 0.0016) and serum asparate aminotransferase (p = 0.0251) than those who had the focal pattern. FIL in 20 patients (14%) imposed a difficulty in making an appropriate diagnosis, led to inaccurate impressions, or forced unnecessary invasive or non-invasive investigations. We conclude from our large series of patients that FIL is not uncommon in hospital practice and among those at risk should always be considered as an appropriate diagnosis.
Br J Radiol 1992 Sep
PMID:Fatty infiltration of the liver: analysis of prevalence, radiological and clinical features and influence on patient management. 139 13

Hepatic veno-occlusive disease (VOD) is the most common life threatening complication of preparative-regimen-related toxicity for bone marrow transplantation (BMT). The frequency of VOD varies greatly, from 1-2% in centers performing pediatric BMT for thalassemia to over 50% in some centers doing BMT for hematologic malignancy. The term liver toxicity syndrome is a clinicopathologic definition which encompasses the range of histopathology within the hepatic venules and surrounding sinusoids and hepatocytes. These histologic abnormalities are statistically associated with a clinical syndrome of jaundice, ascites, and painful hepatomegaly developing early post-transplant. Newer modalities which may aid accuracy are transvenous liver biopsy along with determination of the gradient between the wedged and free hepatic venous pressures, and measurement of blood coagulatory components, particularly protein C levels. Analyses of clinical risk factors for VOD are confounded by lack of a clear hierarchy of risk when comparing heterogeneous patient populations, the methods of patient selection and choice of controls, and whether analysis is univariate or multivariate. Prospective multivariate analyses indicate that the risk of developing liver toxicity is independently correlated with intensity of conditioning therapy, pre-transplant viral hepatitis, use of antimicrobial therapy with acyclovir, amphotericin, or vancomycin (reflecting fever), and mismatched or unrelated allogeneic marrow grafts. These analyses plus morphologic and biochemical data support the hypothesis that VOD is caused by cytoreductive injury to hepatocytes and endothelium in zone three of the liver acinus, and in turn strongly influenced by factors which induce the release of tumor necrosis factor-alpha (TNF-alpha) leading to enhancement or activation of coagulation with obstruction of hepatic sinusoids and venules. Pharmacokinetic measurements of busulfan as a conditioning agent demonstrate a correlation between high steady-state busulfan levels and liver toxicity and suggest that safer and/or more efficacious plasma busulfan concentrations can be obtained by making individual dose adjustments and by changing the schedule of administration. Conservative therapy of severe VOD, including the use of peritoneal-pleural shunts for relief of ascites, is unsatisfactory. Results from prophylactic studies aimed at preventing VOD by heparin or prostaglandin E1 indicate considerable differences with toxicity and efficacy. Use of the TNF-alpha blocker, pentoxifylline, has also shown promise in lessening VOD. A statistical model which predicts patients likely to have an unfavorable outcome from VOD has been used to select premorbid patients for promising new therapeutic modalities, such as recombinant tissue plasminogen activator.
Bone Marrow Transplant 1992 Sep
PMID:Hepatic veno-occlusive disease--liver toxicity syndrome after bone marrow transplantation. 142 75

Twenty-one bone marrow transplantation (BMT) patients were studied prospectively to determine the prevalence of sonographic hepatobiliary abnormalities and to determine if these abnormalities were associated with hepatic venocclusive disease (VOD). Baseline US was performed in all patients prior to chemoradiation therapy, with follow-up ultrasound (US) examinations at the time of BMT and 14 days and 28 days after BMT. Sonograms were reviewed for the presence of ascites, gallbladder wall thickening, hepatosplenomegaly, hepatofugal flow, hepatic vein compression, increased periportal echogenicity, and increased hepatic echotexture. The baseline scans showed 13 of 21 patients (62%) with abnormalities prior to BMT. Within 2 weeks after BMT, serial US showed interval development of hepatomegaly in five patients (three with VOD and two without), gallbladder wall thickening in one (with VOD), hepatic vein compression in two (both with VOD), hepatofugal flow in one (without VOD), and ascites in one (with VOD). US scans obtained 4 weeks after BMT in 15 of the 21 patients showed even fewer new abnormalities. No sonographic finding was strongly associated with VOD.
Radiology 1992 Sep
PMID:Venocclusive disease of the liver: prospective study of US evaluation. 150 50

Preoperative evaluation should focus on techniques that either preclude surgery entirely, alter the planned operation either preoperatively or intraoperatively, or suggest the need for preoperative adjuvant therapy. The presence of a synchronous colonic neoplasia should be excluded by colonoscopy or air-contrast barium enema combined with sigmoidoscopy. A preoperative carcinoembryonic antigen level provides useful prognostic information. Abdominal computed tomography (CT) or hepatic ultrasonography should be done in patients with hepatomegaly or abdominal liver function tests. Those with rectal cancer should undergo a careful digital palpation done by an experienced observer combined with rigid proctosigmoidoscopy. Intrarectal ultrasonography may provide more objective data in regard to the extent of the local disease. If preoperative radiation therapy is planned, abdominal CT is appropriate to exclude extrapelvic disease that would preclude such a strategy. The benefit of routine preoperative CT in patients with otherwise resectable colonic or rectal cancer remains unclear.
Cancer 1992 Sep 01
PMID:Preoperative evaluation of patients with primary colorectal cancer. 151 80

Early trials of allogeneic marrow transplantation for homozygous thalassemia were disappointing in patients older than 16, with four of six patients dying early of graft-versus-host disease-related complications, one patient dying at 9 months of infection due to graft failure, and one dying at 6 years of recurrent thalassemia. Three classes of risk could be identified in analyses of results of transplantation in younger patients using the criteria of degree of hepatomegaly, the presence or absence of portal fibrosis, and a history of adequate or inadequate chelation therapy. Patients for whom all three criteria were adverse constituted a very high risk group (class 3) for marrow transplantation. On the basis of these analyses, a conditioning regimen was designed that yielded superior results for class 3 patients under 17 years of age. Most patients older than 16 years presenting for transplantation have disease characteristics that place them in class 3 and, because of the improved results with the new class 3 regimen in younger patients, a study was designed to treat patients older than 16 years using treatment regimens assigned on the basis of disease class. Twenty patients were treated using this protocol and, with a minimum follow-up of 9 months, there have been three early deaths, one patient has recurrent thalassemia, and 16 patients are alive disease-free. The actuarial probabilities of survival, disease-free survival, and rejection are 0.85, 0.80, and 0.05, respectively, with a survival plateau extending from 6 months to 3 years. Marrow transplantation is a reasonable option for adults with progressive thalassemia who have suitable donors.
Blood 1992 Sep 15
PMID:Bone marrow transplantation in adult thalassemia. 152 Aug 85

A patient with cystic kidney disease of adult onset and severe cystic hepatomegaly is presented. The patient was severely disabled solely by her abdominal bulk. Simultaneous liver and renal transplantation was undertaken successfully.
Q J Med 1991 Sep
PMID:Massive cystic hepatomegaly in a female patient with polycystic kidney disease treated by combined hepatic and renal transplantation. 175 77

After the intraperitoneal injection of corpuscles of C. burnetii antigen (Ag), phospholipid (PL), and sediment obtained after the extraction of PL from Ag with chloroform-methanol (CM) slight leukocytic reaction developed in the peritoneum on day 1, and on day 2 it could be observed in the liver and in the spleen. Ag induced the most pronounced morphological changes. In the spleen they were manifested by the activation of T- and B-dependent zones of white pulp from day 2 and by the pronounced hyperplasia of reticular cells and macrophages, leading to splenomegaly, by days 7-14. Simultaneously lymphoid-macrophagal granulomas and hepatomegaly developed in the liver. By days 7-14 the foci of necrosis in the liver were caused by the thrombosis of portal veins and were not registered after the injection of PL and CM (and earlier also in experiments with Ag in doses of 0.1-0.3 mg).
Zh Mikrobiol Epidemiol Immunobiol 1991 Sep
PMID:[The host reaction to the administration of different components of Coxiella burnetii]. 175 30

Four patients with moderately severe type I Gaucher disease were treated with commercially available mannose terminated glucocerebrosidase (Ceredase; Genzyme, Boston, MA) for up to 13 months. The enzyme was administered at the rate of three to four times weekly at one fourth the total recommended dosage, greatly decreasing the cost. Marked regression of hepatomegaly and improvement in liver function tests, peripheral blood counts, and serum angiotensin-converting enzyme levels were documented. The two patients with pulmonary involvement manifested improvement in pulmonary function tests. Skeletal disease remained unchanged.
Blood 1991 Sep 01
PMID:Enzyme replacement therapy for Gaucher disease. 187 85

The course of liver involvement during the first three weeks of typhoid fever was studied in 20 patients. Previous studies of liver involvement in typhoid fever have not considered the time course of changes. In this study, hepatomegaly was found during the 2nd or 3rd wk more often than in the 1st wk (36% vs. 11%), whereas jaundice was detectable in 9% of patients after the 1st wk, but never before. Alkaline phosphatase, AST, and ALT were raised in 100%, 100%, and 91% of cases, respectively, during the 2nd and 3rd wk but during the 1st wk, only 11%, 89%, and 56% had mild increases. This study shows that, although the clinical picture of hepatitis is unusual, liver involvement is invariably present after the 1st wk, and should not be considered as a complication, but as a feature of the disease.
Am J Gastroenterol 1991 Sep
PMID:The liver in typhoid fever: always affected, not just a complication. 188 3


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