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 present a report of a pregnancy complicated by chronic schistosomiasis. A.H. is a 22 year old G1 Filipina, referred at 34 weeks gestational age for evaluation of hypersplenism and progressive thrombocytopenia. Physical exam was remarkable for hepatosplenomegaly. Laboratory evaluation of hepatic, renal, hematologic, autoimmune, or infectious disease etiology or abnormality was negative. Ultrasound revealed a normal fetus, an enlarged spleen, dilated splenic and hepatic vasculature and an enlarged liver with periportal fibrosis. Abdominal computed tomography revealed subcapsular calcifications, dilated ducts, and periportal fibrosis. Esophagogastrojejeunoscopy showed no varices. The presumptive diagnosis of hypersplenism secondary to chronic hepatosplenic schistosomiasis was made. Based on pathophysiology and literature review conservative management was elected. Liver functions, clotting functions, and platelet counts were monitored closely. Intense antepartum fetal monitoring was performed. Platelet counts remained between 30 and 40K. The patient went into labor at 38 3/7 weeks and delivered a 3148 g female Apgars 8/9 by spontaneous vaginal delivery. Evaluation of the placenta was negative for ova. By 2 weeks postpartum platelet counts had increased to the 60-70 k range, and a rectal biopsy, positive for S. japonicum ova was performed. Treatment with three doses of Praziquantel 20 mg/kg q8h was completed prior to her return to the Philippines.
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PMID:Hepatosplenic schistosomiasis in pregnancy: report of a case and review of the literature. 212 Nov 52

The tropical splenomegaly syndrome (TSS) is characterized by massive splenomegaly with hypersplenism, moderate hepatomegaly, and lymphocytic infiltration of the hepatic sinusoids. In previous reports this syndrome has been shown to be a consequence of a disordered immunologic response of the host to malarial infection. Treatment with antimalarial drugs has resulted in a decrease in malarial antibody titers and a reduction in splenic size. We report a child who had TSS associated with cytomegalovirus infection rather than malaria. Our results suggest that TSS may be precipitated by a variety of infections producing chronic antigenic stimulation and perhaps by autoantigenic stimulation as well.
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PMID:Tropical splenomegaly syndrome associated with cytomegalovirus infection. 254 Apr 53

Three cases of a syndrome featuring massive splenomegaly, gross generalized lymphadenopathy, and moderate hepatomegaly are reported. Spleen weights ranged from 800 to 2400 g. Gradual depletion of lymphoid germinal centers, and prominent infiltration of the splenic and lymph node cords with plasma cells, immunoblasts and actively dividing B cells were the most distinctive histological features. The liver in two cases showed portal infiltrates. A marked hypergammaglobulinemia, a decrease in blood cholesterol level and hematological abnormalities related to hypersplenism were observed. The condition begins early in life and runs a chronic course, of up to 25 years. There was a family history in only one instance. Since there was no generalized immunodeficiency nor local depletion of T cells or dendritic reticulum cells, a failure in the local regulation of the immune response and possible cytokine production is postulated. This condition underlines the pivotal role of the local organization of the germinal centers in cellular cooperation and in the carrying out and regulation of the immune response.
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PMID:The disappearance of germinal centers in chronic lymphadeno-hepato-splenomegaly syndrome in childhood: report of three cases. 271 99

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.
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PMID:Late symptoms in infantile cystinosis. 315 26

Gaucher's disease involves the liver, the spleen, and the bone. Liver-spleen and bone scintigraphy were used during an 8-yr period to evaluate changes caused by this disease. Patients were investigated with a liver-spleen scan for abdominal pain, mechanical discomfort, enlarged liver or spleen on physical examination, abdominal mass, abnormal liver function tests, and symptoms of hypersplenism. Fourteen liver-spleen scans were performed in nine patients. Liver scintigraphy showed various degrees of enlargement and inhomogeneous uptake. In two patients focal defects were detected. In one, focal defects were due to liver involvement with Gaucher's disease, but in the other they were caused by metastatic pancreatic carcinoma. The study was also useful in detecting splenic infarction and in following enlargement of the spleen after partial splenectomy. The main indication for bone scintigraphy in six patients was bone pain. This was found to be caused by either aseptic necrosis of the head of the femur, bone infarction, pathological fractures, or osteomyelitis. Loosening after total hip replacement was ruled out in three patients and missed in one patient. Scintigraphy appears to be a simple, sensitive test for evaluation of the liver, spleen, and bony skeleton in patients with symptomatic Gaucher's disease.
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PMID:Scintigraphic findings in Gaucher's disease. 376 Sep 80

Hepatic tuberculosis was confirmed in 96 patients presenting with the features of liver disease, only 14 of whom had other concomitant hepatic pathology. Although respiratory symptoms occurred in 74 per cent of cases, these were overshadowed by the abdominal manifestations. The latter most frequently included right hypochondrial pain, abdominal distension, firm tender hepatomegaly, splenomegaly and ascites. Icterus was observed in 11 cases (only one of whom had concurrent hepatic pathology) and liver failure was found in 10 patients. A surgical presentation occurred in three patients. Four of 15 patients with pancytopenia were noted to have hypersplenism. Abnormalities in coagulation were noted in 26 patients (24 with low prothrombin index and two with moderately raised fibrinogen degradation products). The characteristic serum profile included hyponatraemia (64 per cent of cases), raised alkaline phosphatase (83 per cent) and gamma glutamyl transferase (77 per cent), hypoalbuminaemia (63 per cent) and hypergammaglobulinaemia (83 per cent). Transaminase levels were moderately elevated in 78 per cent of cases. Hepatic imaging techniques were frequently misleading. Chest radiographs aided the diagnosis but were normal in 25 per cent of cases. Histologically, acid fast bacilli, caseation and granulomas were seen in 9, 83 and 96 per cent of cases respectively. Adverse prognostic features included age (below 20 years), miliary TB, coagulation defects and the presence of predisposing factors; these were of value in selecting appropriate therapy. The overall mortality was 42 per cent. Liver biopsy was the most useful aid to correct diagnosis which was suspected clinically in only 47 per cent of cases.
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PMID:Tuberculosis hepatitis: a clinical review of 96 cases. 651 2

Thirteen patients underwent splenectomy for Gaucher's disease. All patients were Jewish; 12, of Ashkenazi descent, had the chronic (type 1) form, and one child, of Sephardic (Persian) origin, probably had the intermediate (type 3) form. Hypersplenism was the indication for surgery in 11 patients, mechanical problems in the remaining two. The weight of the resected spleens ranged from 1.06 to 13 kg. Following surgery, hypersplenism (thrombocytopenia in particular) was improved, and the mechanical disturbances were relieved in all patients. There were no deaths and no morbidity related to the operative procedure. Long-term follow-up demonstrated progressive hepatomegaly without evidence of hepatic dysfunction in any of the patients. Bone marrow involvement manifested by osteoarticular complications appeared in five patients. Splenectomy is, we believe, a safe mode of treatment for type 1 Gaucher's disease.
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PMID:The role of splenectomy in Gaucher's disease. 687 May 24

We report a rare case of tricuspid regurgitation due to nonpenetrating chest trauma 33 years previously. A 79-year-old man suffered a blunt trauma due to a piece of wood at work in 1958. He suffered multiple rib fractures on the right side and was admitted. Since then, he began having shortness of breath on exertion and was treated with medication. The patient was transferred to the Division of Cardiology, Hakodate National Hospital in 1984. A chest x-ray film revealed a marked cardiomegaly. Cardiac catheterization showed severe tricuspid regurgitation. Hepatomegaly and pancytopenia was observed. He was readmitted because of general fatigue in July 1991. Two-dimensional echocardiography demonstrated systolic excursion of septal and posterior tricuspid leaflets with ruptured chordae tendineae into the right atrium, and a remarkably enlarged right ventricule, right atrium and vena cava interior. Cardiac catheterization was performed. The right atrial pressure-wave form resembled the right ventricular pressure recording (ventricularization of the atrial pressure). Right ventricular cineangiography revealed severe tricuspid regurgitation, grade 4. Laboratory data showed pancytopenia. Thrombocytopenia progressed (3 x 10(4)/mm3), and a hemorrhagic tendency developed. The liver edge was palpable 4 finger breadths below the right costal margin. Pancytopenia due to congestive hepatomegaly and hypersplenism would have complicated this case.
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PMID:[A case of long-standing isolated traumatic tricuspid regurgitation with remarkably dilated right cardiac chambers and pancytopenia]. 828 48

Splenomegaly was studied at a municipal teaching hospital in an 11-year retrospective review. The 170 patients were classified into six diagnostic groups. The associated clinical and laboratory features were tested for statistical association (X2), to determine predictive values. Hepatic diseases caused 36% of the splenomegaly; hematologic, 35%; infectious diseases (ID), 16%; inflammatory, 5%; primary splenic, 4%; and other, 3%. The acquired immunodeficiency syndrome (AIDS) occurred in 54% of patients with ID. Hematologic diseases were significantly associated (P < 0.01) with massive splenomegaly, left upper quadrant (LUQ) abdominal tenderness, and all blood "cytoses." The most common disease with massive splenomegaly was myelofibrosis. Surprisingly, hepatic diseases caused 29% of massive splenomegaly. Hepatic diseases were significantly associated (P < 0.01) with hepatomegaly, abnormal liver-function tests (LFT), and blood "cytopenias." Compared with previous reports, both congestive heart failure and endocarditis now rarely cause splenomegaly. All blood "cytopenias" had highly significant associations (P < 0.01) only with hepatic diseases, which suggests that hypersplenism remains a useful concept for the splenomegaly of liver disease.
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PMID:Splenomegaly at a United States County Hospital: diagnostic evaluation of 170 patients. 885 64

Splenomegaly and massive splenomegaly were diagnostically evaluated retrospectively at Stanford University Hospital in 147 patients over 8 years and compared to the nearby county hospital (Santa Clara Valley Medical Center; VMC) in 170 inpatients over 11 years. Hematologic diseases at Stanford (data for VMC in parentheses) occurred in 66% (35%; p < 0.001) of the patients with splenomegaly and in 84% (54%; p < 0.001) of those with massive splenomegaly. Hepatic diseases occurred in 9% (36%; p < 0.001) of the patients with splenomegaly and in 5% (29%; p < 0.001) of those with massive splenomegaly. Splenectomy was performed in 71% of the patients at Stanford and in 9% of those at VMC (p < 0.001). The combined Stanford-VMC series showed significant associations (p < 0.01): for hematologic diseases with massive splenomegaly, lymphadenopathy, and blood cytoses; for hepatic diseases with hepatomegaly, cytopenias as hypersplenism, and abnormal liver function tests, and for infectious diseases with fever.
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PMID:Splenomegaly at a university hospital compared to a nearby county hospital in 317 patients. 1052 11


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