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

Forty-three patients with spontaneous bacterial peritonitis (SBP) between 1973 and 1978 were identified. Criteria for SBP included a positive ascites culture and polymorphonuclear cell concentration greater than 250 cells per mm3. Chronic liver disease was documented by varices in 91%, severe histologic fibrosis or cirrhosis in 94%, splenomegaly in 91%, and past hospitalization for liver disease in 57% of the patients. SBP was detected within 7 days of admission in 17 patients (40%) and within 35 days in 38 patients. Single organisms were isolated from 38 patients and multiple organisms from 5 patients. Twenty-six of 43 patients survived the episode of SBP, but only 13 survived the hospitalization. Analysis of the survival curve from the onset of SBP revealed a rapid death rate and a slow death rate set of patients. Rapid death (less than or equal to 7 days from SBP onset) correlated with a lack of prior hospitalization for liver disease (p less than 0.001), hepatomegaly (p less than 0.001), increased serum bilirubin (p less than 0.005), serum creatinine (p less than 0.05), and peripheral white blood cell concentrations (p less than 0.05). Survival during hospitalization was associated with prior hospitalization with liver disease (p less than 0.001) and chills during the episode of SBP (p less than 0.001). The 43 patients were divided into Group 1 patients on the basis of a serum bilirubin greater than 8 mg% and/or serum creatinine greater than 2.1 mg%; Group 2 patients had lower values. Survival was greater in Group 2 patients with advanced, relatively quiescent liver disease compared to Group 1 patients for both the episode of SBP (91 vs. 29%; p less than 0.001) and for hospitalization (50 vs. 9%; p less than 0.05). Death in Group 2 patients was related to inadequate antibiotic therapy (p less than 0.05), nonhepatic factors, and new onset of renal failure. Although SBP in the setting of severe acute liver injury has a dismal prognosis, SBP with minimal acute liver injury has a relatively good prognosis for hospital survival even with advanced chronic liver disease. Long-term survival is also possible since 4 of 9 patients with prolonged follow-up have survived 3 years.
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PMID:Spontaneous bacterial peritonitis. 709 41

We report the clinical outcome of 105 essential mixed cryoglobulinemia (EMC) patients with renal involvement collected throughout 25 years in three renal Units of Milan. The median follow-up was 72 months since renal biopsy and 131 months since the clinical onset of EMC. Patient survival was 49% at 10 years after renal biopsy. Forty-two patients died primarily from cardiovascular and liver disease or infection, whereas 15 patients developed chronic renal failure. Two patients had a complete remission of the disease while 15 had a remission only of renal signs. Thirty-one patients are alive with persistent renal and extrarenal manifestations. Anti-HCV antibodies were retrospectively detected in 34 patients and were present in 85% of them. This variable was not included in the statistical evaluation. At multivariate analysis, age older than 50 years, purpura, splenomegaly, cryocrit levels higher than 10%, C3 plasma levels lower than 54 mg/dl, and serum creatinine higher than 1.5 mg/dl were independent risk factors for death or dialysis. In conclusion, several factors may influence the outcome of patients with EMC nephritis. Markers of disease activity and an impaired renal function can herald a bad prognosis. It should be stressed, however, that only a minority of patients eventually develop renal failure, probably because in the most severe cases patients die earlier.
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PMID:Long-term predictors of survival in essential mixed cryoglobulinemic glomerulonephritis. 772 49

We prospectively studied 50 Vietnamese patients with blackwater fever (BWF). All patients had fever and hemoglobinuria, 40 (80%) were jaundiced, 25 (50%) had hepatomegaly, 15 (34%) had splenomegaly, and 9 (18%) had hepatosplenomegaly. Twenty-one patients (42%) had impaired renal function, with creatinine clearances of < 50 mL/min/m2; however, only four (8%) developed oliguric renal failure, three (6%) of whom required dialysis. Forty-four patients (88%) developed anemia, which was severe (hematocrit, < 20% in 32 (64%). One patient died, representing a death rate for this once-feared disease that is considerably lower than that reported by earlier investigators. BWF was associated with quinine ingestion in 28 patients (56%), glucose-6-phosphate dehydrogenase (G6PD) deficiency in 27 (54%), and concurrent malaria infection in 16 (32%). There was no statistically significant difference in the severity of BWF associated with each of these three factors, as assessed by creatinine clearance and the hematocrit value on admission and by the number of units of blood transfused. There was considerable overlap in the occurrence of G6PD deficiency, quinine ingestion, and malaria, suggesting that these factors may interact and that it may not be justifiable to regard hemoglobinuria caused by G6PD deficiency as a separate syndrome.
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PMID:Blackwater fever in southern Vietnam: a prospective descriptive study of 50 cases. 940 15

In contrast with the well-recognized membranous obstruction of the inferior vena cava, short-length hepatic vein stenoses are not well-recognized causes of hepatic venous outflow block. The aim of this study was to ascertain the prevalence, causes, manifestations, and outcome of short-length hepatic vein stenoses. We performed a retrospective study of patients with short-length hepatic vein stenosis among 86 patients with hepatic venous outflow block who were seen between 1970 and 1992. There were 25 patients with short-length hepatic vein stenosis. A thrombogenic condition was identified in 14 patients (56%). The lesions of the accompanying hepatic veins in these patients were variable (short-length stenoses, thromboses, or nonspecific changes) and similar to that seen in patients without short-length hepatic vein stenosis. In 3 necropsied cases, the venous lesions were suggestive of fibrous sequela of prior thromboses. In patients with short-length hepatic vein stenosis, splenomegaly (28% vs. 55%, P < .05) and hypersplenism were significantly less common; serum transaminase (P < .001) and creatinine levels (P < .02) were lower, prothrombin was higher (P < .001), and 5-year survival was significantly better (Kaplan-Meier estimates: 80% vs. 50%, P < .05). In patients with hepatic venous outflow block, short-length hepatic vein stenosis is a common lesion that appears to be the sequela of localized thrombosis. Long-term anticoagulation and percutaneous angioplasty (with or without stenting) are potentially applicable in these lesions. The long-term results of these treatments merit further evaluation.
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PMID:Hepatic venous outflow block caused by short-length hepatic vein stenoses. 909 81

This paper describes the clinico-pathological parameters measured in dogs that were vaccinated against Babesia canis using soluble parasite antigens (SPA) and then challenged. The packed cell volume (PCV) and the plasma creatinine value decreased immediately after challenge. Actual PCV values could be predicted in the first 5-6 days of the infection, assuming that creatinine values were modulated by increase of plasma volume. This association no longer existed after that time, and observations indicated splenic involvement in reduction of numbers of circulating erythrocytes. The anaemia due to B. canis infection appears to be the result of a multifactorial process including plasma volume increase, erythrocyte retention in the spleen and erythrocyte destruction, partly due to parasite proliferation. Vaccination limited the reduction of PCV values, and the development of splenomegaly. Differences in protection between vaccinated and control animals became apparent 6 days after infection, when a memory immune response becomes operative, and the onset of recovery of vaccinated animals correlated with the onset of antibody production against SPA.
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PMID:Vaccination of dogs against Babesia canis infection. 947 90

The authors performed a late evaluation of a distal splenorenal anastomosis minimum of five years following operation on 13 patients with schistosomiasis of the compensated liver-splenic type. The study of the anastomosis had been proven patent when the evaluation took place. Each patient underwent clinical, laboratorial, endoscopic and electroencephalographic assessment. The results demonstrated that no patient had shown any sign of recurrence of upper gastrointestinal hemorrhage. Among the endoscopic aspects, esophageal varices disappeared in 46.1% of the cases. There was reduction in the number, extent and volume of esophageal varices in 46.1%, 38.4% and 53.8% of the cases. Gastric varices disappeared in 91.6% of the cases. Only one patient (7.6%) had shown clinical and electroencephalographic signs of hepatic encephalopathy in the late final evaluation (non-significant). Only one patient (7.6%) had shown late postoperative ascites (non-significant). There were no significant alterations in serum levels of sodium, potassium, urea and creatinine in all the 13 patients. The values of indirect serum bilirubin increased in 92.3% of the patients. There was regression of splenomegaly in all 13 patients, as well as a significant improvement in their hematological values. There were no significant changes in the serum levels of aspartate aminotransferase and alanine aminotransferase or in the activity of the plasma prothrombin. The authors concluded that the distal splenorenal anastomosis became a protection factor against upper gastrointestinal hemorrhage and led to long-term improvement in the endoscopic aspects of esophagogastric varices, a significant improvement in the laboratorial aspects of hypersplenism and a marked reduction of splenomegaly with no significant changes in the hydroelectrolytic metabolism, renal function and hepatic function and had not compromised, long term, the quality of life of the majority of patients.
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PMID:Late clinical, biochemical, endoscopic and electroencephalographic evaluation of patients with schistosomal portal hypertension treated with distal splenorenal shunt. 970 17

Between January and December 1996, we observed 64 children (mean age 8.3 years range 4.2 to 11.2 years) who required dialysis for severe acute renal failure secondary to Falciparum Malaria. All received anti malarial therapy and other supportive therapy as well as peritoneal dialysis. Out of these 28 died (43.8%). The children who died (Group I) compared to those who survived (Group II) differed significantly in age (mean +/- SD) (7.2 +/- 1.3 years vs. 9.2 +/- 2.1 years P < 0.05), plasma creatinine at presentation (645 +/- 104 mumol/L vs. 438 +/- 87 mumol/L P < 0.05), plasma bilirubin (2.1 +/- 0.3 mg/dL vs. 1.2 +/- 0.2 mg/dL P > 0.02) systolic BP (50 +/- 11 mmHg vs. 90 +/- 12 mmHg P0 < 0.01), diastolic BP (20 +/- 4 mmHg vs. 60 +/- 9 mmHg P < .01) .Hb level 5.3 +/- 0.4 g/dL vs. 8 +/- 1.3 gm/dL P < .02), time from diagnosis to referral (5.3 +/- 1.3 days vs. 8.9 +/- 2.1 days P < .05) and urine output (200 +/- 49 mL/24 h vs. 600 mL +/- 131 mL P < .01). There was no significant difference in gender, alanine transaminase (ALT) level, degree of fever, plasma Na or plasma K. Diarrhea was present in 29% of the children who died and in only 11% of those who survived (P > 0.05) and splenomegaly was found in 3% and 18% respectively (PO > .05).
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PMID:Prognosis of malaria associated severe acute renal failure in children. 1004 18

Many patients with type II mixed cryoglobulinemia have been shown to be infected with hapatitis C virus (HCV). Therefore, interferon-alfa has become the first choice of treatment for patients with HCV-associated cryoglobulinemia. However, the disease often relapses after the discontinuation of interferon therapy. The long-term effect of interferon therapy is controversial. Therefore, a more effective therapy needs to be developed. A 62-year-old Japanese woman was admitted to our hospital for the examination of abnormal liver function tests, severe edema, and purpura in her lower extremities. Glomerulopathy secondary to HCV-related cryoglobulinemia was suspected. Her serum creatinine was increased to 2.1 mg/dL. Interferon therapy was considered initially. However, because of pancytopenia caused by liver cirrhosis and splenomegaly, splenectomy was performed in February 1997, before the start of interferon therapy. Renal biopsy specimen taken at the time of the splenectomy showed typical cryoglobulinemic glomerulonephritis. Gradually, after surgery, the patient's thrombocytopenia and anemia improved, her proteinuria and hematuria were decreased, her cryocrit dropped from 15% to 5%, the Ccr increased from 21.1 mL/min to 48.8 mL/min, and the purpura in her lower extremities disappeared. A repeat renal biopsy performed in May 1998 showed marked histological improvement. Splenectomy is not widely accepted as a treatment for cryoglobulinemia. Our case suggests the possibility that the monoclonal-IgM component of the type II cryoglobulin may be formed in the spleen. In conclusion, splenectomy may be an effective therapy for cryoglobulinemia in patients with HCV-positive liver cirrhosis and pancytopenia secondary to splenomegaly.
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PMID:Splenectomy may improve the glomerulopathy of type II mixed cryoglobulinemia. 1084 34

An increase of angiogenesis has been shown in idiopathic myelofibrosis with myeloid metaplasia (MMM) by microvessel density count method but evaluation of circulating angiogenic factors is still incomplete. In 31 patients affected by MMM and in 12 healthy subjects we evaluated the serum levels of VEGF (vascular endothelial growth factor) and correlated VEGF with clinical and laboratory features of disease. We found that MMM patients had circulating VEGF concentrations much higher than controls (Median 1208 ng/ml vs 138 ng/ml, P < 0.0001). No correlation was found between VEGF and Hb, WBC, PLT, LDH, creatinine, bone marrow cellularity, fibrosis, splenomegaly, hepatomegaly, and therapy. However, in the subgroup of patients with a normal or low VEGF concentration, a direct correlation between VEGF and platelet count (r = 0.90, P = 0.002) was detected. Moreover, patients with a platelet count < 300 x 10(9)/l had VEGF serum levels lower than patients with a higher PLT count (median VEGF 864 vs 1557 pg/ml, P = 0.001). In six patients and in eight controls we also had the opportunity to measure VEGF in the plasma and we calculated that VEGF concentration was much higher in platelet-rich than in platelet-poor plasma and that platetets of MMM patients contained four times more VEGF than those of healthy controls. These results indicate that VEGF is overproduced in MMM, thus confirming an increased angiogenic activity. Platelets are probably a major source of VEGF in MMM but not the only one.
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PMID:Elevated vascular endothelial growth factor (VEGF) serum levels in idiopathic myelofibrosis. 1141 86

Primary plasma cell leukemia (PPCL) is a rare form of disease accounting for 1-2 percent of myelomas. Between September 1990 and November 2000, among 540 patients with myeloma studied, 24 fulfilled the criteria of PPCL (4.4 percent). We found high frequencies of female patients (62 percent), Bence Jones proteinuria (79 percent), anemia (88 percent), bleeding (54 percent), confusional syndrome (42 percent), weight loss (71 percent), hepatomegaly (25 percent), splenomegaly (21 percent), leukocytosis (62 percent), and thrombocytopenia (71 percent). High serum levels of creatinine, calcium, lactate dehydrogenase (LDH), and beta(2)-microglobulin were detected in 50 percent, 37 percent, 58 percent, and 71 percent, respectively. Four patients were treated with vincristine, melphalan, cyclophosphamide, prednisone, and adriamycin (VMCPA), 12 with vincristine, adriamycin, and dexamethasone (VAD), and 8 with M-80 (oral melphalan 80 mg/m(2) plus dexamethasone 40 mg/m(2)). There was a trend toward lower values of Karnofsky score (P=0.07) and higher values of LDH (P=0.2) in the VAD group. Other clinical characteristics were comparable among the three groups. Complete plus partial responses were achieved in one and six patients treated with VMCPA and M-80, respectively. All patients treated with VAD failed to respond to treatment. Patients receiving the M-80 regimen experienced higher platelet toxicity (P=0.05), vomiting (P<0.0003), and mucositis. Also, the need for red blood cell transfusions was higher in the M-80 group. Median overall survival was 60 days. Overall survival was better in patients achieving complete or partial response. In conclusion, our study illustrates that intermediate doses of melphalan plus dexamethasone are an effective chemotherapy regimen for this aggressive disease. Response to treatment is the only prognostic factor for survival in these patients.
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PMID:Intermediate doses of melphalan and dexamethasone are better than vincristine, adriamycin, and dexamethasone (VAD) and polychemotherapy for the treatment of primary plasma cell leukemia. 1218 4


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