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)

The clinical, haematological and biochemical features of 50 Sudanese patients with sickle cell disease (SCD) were determined. Of 23 patients with complete family data, 21 had sickle cell anaemia (homozygous HbSS), 2 had sickle-cell/beta+thalassaemia but none had sickle cell/beta Othalassaemia. The remaining 27 patients had HbSS phenotype. 84% of patients were from the Baggara tribe in western Sudan, where HbS is a natural extension of the west African HbS belt. 21 patients were children under 2 years old; 19 were 3-10 years old; and the remaining 10 were over 10 years old. Young patients presented mainly with painful vaso-occlusive crisis, severe anaemia, hand and foot syndrome, fever, underweight, malnutrition and various infectious diseases. All patients had mild to moderate cardiac enlargment; 42% had a moderately enlarged spleen but only 10% had an enlarged liver; 20% had infarctive lesions of long bones and another 8% had Salmonella osteomyelitis. Leg ulcers, priapism, enuresis and cholelithiasis were not observed. Patients had a mean haemoglobin concentration of 7.3 g/dl; reticulocyte count of 15.1%; serum bilirubin of 2.1 mg/dl; HbA2 level of 2.8% and HbF of 7%. Thus, the observed pattern of SCD in Sudan is comparable to the severe type described for Africans and not comparable to the benign form found in Shiite Moslem Arabs of Saudi Arabia. 6 adults with mild SCD had HbF levels below 5%. Amelioration of the disease, therefore, does not seem to be related to HbF levels; nor was it possible to relate it to high levels of erythrocyte 2,3-diphosphoglycerate.
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PMID:Sickle cell disease in Sudan. 245 19

The relationships between S. haematobium, hookworm, malaria, hemoglobin level, splenomegaly, and hepatomegaly before and 8 months after treatment with a single dose of metrifonate or praziquantel were studied in Kenyan primary schoolchildren in an area where anemia, S. haematobium, and hookworm are common and malaria is holoendemic. Children with light to moderate S. haematobium infection were examined (Exam 1), assigned at random to groups receiving placebo (PL, n = 104), metrifonate (MT, n = 103, dose 10 mg/kg body weight) or praziquantel (PR, n = 105, dose 40 mg/kg body weight), treated, and examined 8 months later (Exam 2). At Exam 2, 62% of the MT group still passed S. haematobium eggs vs. 13% in the PR group. Egg reduction rates were substantial in both groups, but greater in the PR group; geometric mean egg counts in both groups were very low. Prevalence and intensity in the PL group had not changed between exams. Hookworm egg counts were significantly reduced in the MT group (59% egg reduction rate); malarial infection had increased in all 3 groups, presumably due to the long rainy season between exams. Hookworm egg count was the most significant predictor of initial hemoglobin level, followed by S. haematobium egg count and presence of malarial infection. Treatment with a single dose of MT or PR can produce substantial decreases in S. haematobium infection 8 months later.
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PMID:Single dose metrifonate or praziquantel treatment in Kenyan children. I. Effects on Schistosoma haematobium, hookworm, hemoglobin levels, splenomegaly, and hepatomegaly. 250 1

On June 11, 1986, a 70-year-old man was introduced to our hospital because of an elevated AFP and hepatomegaly. He was diagnosed as having an HCC in the left medial segment and a transcatheter arterial embolization (TAE) was able to reduce his AFP level. In December, 1986, repeated tarry stool was noted, and he was readmitted to hospital on January, 28, 1987, because of severe anemia. An ordinary X-ray revealed an abnormal gas shadow in the right upper abdomen. A subsequent endoscopic examination showed a tumoral mass protruding into the duodenal lumen through a duodenal perforation. After death an autopsy revealed that the perforation was due to the expansive growth of the tumoral mass to the duodenum.
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PMID:[A case of hepatocellular carcinoma (HCC) with bleeding due to duodenal perforation by the tumor]. 255 30

Bone marrow involvement was seen in 11 percent of patients with Hodgkin's Disease which was determined from pre-treatment biopsy specimens using established histopathologic criteria. Analysis of 32 evaluable patients with marrow involvement showed male preponderance with a peak in fourth decade of life. Twenty four cases (75%) had B-symptoms and 15 (46%) presented within six months of onset of symptoms. On categorizing for clinical staging, 21 (65%) belonged to stage III and IV. Hepatomegaly (greater than or equal to 2 cms) was present in seven cases (21%) and splenomegaly in 13 cases (40%). Mixed cellularity and lymphocytic depletion histopathologic subtypes showed the highest frequency of involvement (21 cases; 65%). Out of 28 cases ESR was raised in 27 cases (96%). Eighteen cases (56%) showed elevated serum alkaline phosphatase levels. Serum copper levels were determined in 14 cases, out of which 12 (85%) showed elevated levels. These parameters along with anemia (hemoglobin of 12 g/dl or less) in 26 cases (81%), correlated well with the disease activity. Only four cases had leukopenia at presentation pointing to no hindrance for aggressive chemotherapy. All cases received minimum of six courses of standard combination chemotherapy with or without local radiotherapy. Sixteen cases (50%) relapsed subsequently and were managed accordingly. A five year follow-up revealed a minimal 31 percent overall survival, and 18 percent of patients were disease free and well since the time of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hodgkin's disease with bone marrow involvement. 259 99

We have analysed 139 consecutive cases (71 males and 68 females) of nutritional megaloblastic anaemia over a period of four and a half years. The majority of these patients belonged to the low socio-economic class and many of them were strict vegetarians. Sixty one percent were in the second and third decades of life. At the time of presentation, 46% had mild hepatomegaly, 42% fever, 34% mild splenomegaly and 20% bleeding manifestations. Of 102 cases in whom the biochemical parameters were available, vitamin B12 deficiency was detected in 76%, folate deficiency in 6.8%, combined B12 and folate deficiency in 8.8%; the remaining 7.8% had normal vitamin levels at presentation. All 139 patients had severe anaemia, 80.5% had thrombocytopenia and 43.8% had neutropenia as well as thrombocytopenia. It appears that during progression (in terms of duration) of megaloblastosis, anaemia is followed by thrombocytopenia and then neutropenia. Infection and bleeding in these patients may be aggravated by impaired functions of neutrophils and platelets, respectively.
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PMID:Pancytopenia in nutritional megaloblastic anaemia. A study from north-west India. 263 48

A thirty year-old man with angio-immunoblastic lymphadenopathy (AILD) is presented. This is the first case recorded from Ethiopia, and few have been reported elsewhere in Africa. The patient presented with generalized lymphadenopathy, fever, weight loss, pruritus, skin rashes, anaemia, hepatomegaly and pulmonary infiltrates. Lymph node histology was typical of AILD. The available literature on this relatively new pathologic entity reveals that little is known about the nature of AILD, and since there is no established therapy, an individualized approach to management is advisable.
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PMID:Angio-immunoblastic lymphadenopathy with dysproteinaemia in an Ethiopian: case report and literature review. 264 18

The clinical and hematological profile of 53 patients in whom primary myelofibrosis (PMF) had been diagnosed during the last 15 years was evaluated. Median age was 64 years (range 17-86). Thirty-five patients were males and 18 females. The most frequent symptoms were associated with the hypermetabolic state, anemia and splenomegaly. The latter was found in 96% of patients, while 83% had hepatomegaly and 9% had lymphadenopathy. Thirty-three patients had anemia at the time of diagnosis. The leukocyte and platelet counts were normal or moderately high in most cases. Myelemia was found in 83% of patients, with circulating erythroblasts in 72%. The most common biochemical abnormalities were the increased serum LDH (84%) and hypocholesterolemia (62%). Bone marrow aspirate was not analyzable in most cases. Bone marrow biopsy showed myelofibrosis in hypercellular phase in 22 patients, myelofibrosis without osteosclerosis in 17, and myelofibrosis with osteosclerosis in 14. The median survival of the series was 3.8 years; 34 patients had died at the time of the analysis. The major causes of death were infection, cardiovascular complications and hemorrhage. In 4 patients the evolution from PMF to acute leukemia was observed.
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PMID:[Primary myelofibrosis: description of a series of 53 patients]. 266 65

The clinical and hematological profile, the response to therapy and the clinical evolution of 29 patients with acute T lymphoproliferative syndromes (ATLS) diagnosed during the last 5 years were evaluated. Mean age was 22.6 (SD 9.5) years. 24 patients were males and 5 females. Lymphadenopathy was present in 25 patients, hepatomegaly in 16 and splenomegaly in 14. Seventeen patients had a mediastinal mass. Anemia was present in 41% of patients and thrombocytopenia in 50%. The mean leukocyte count was 71 x 10(9)/l (SD 93). Bone marrow infiltration was found in 25 patients. The acid phosphatase reaction was positive, centrosomal type, in all cases except two. The immunophenotypic study classified the ATLS patients in the subtypes pre-T (10 cases), cortical thymic (15 cases), and mature thymic (4 patients). The cases with pre-T phenotype had a greater degree of bone marrow infiltration and lower frequency of mediastinal mass than the rest of patients. The rate of complete remissions (CR) was 83%. At the time of closing the study 12 patients had relapsed, with a median time of CR of 9.4 months. In addition, 11 patients had died, with a median survival of the series of 19 months. The results of the present study indicate that, despite some heterogeneity, those cases of ATLS with a more immature immunological phenotype (pre-T) have clinical and biological features more akin to acute leukemia, unlike those with a thymic phenotype (cortical or mature), which would be closer to lymphoblastic lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Acute T-cell lymphoproliferative syndromes. A study of 29 cases]. 269 96

It is known that cigarette smoking induces leukocytosis and increased genetic instability in normal individuals. Therefore, a retrospective review was conducted of 173 patients with chronic myelogenous leukemia to detect a possible influence of cigarette smoking on initial characteristics at the time of presentation and on the course of this disease. Thirty-nine patients (23%) were smoking 5 cigarettes/d or more at time of diagnosis. Cigarette smoking was significantly related to male sex (P = 0.0005) and younger age at diagnosis (P = 0.02) and smokers tended to have lower leukocyte counts (P = 0.07) than nonsmokers. Cigarette smoking was significantly associated with early blast crisis (P less than 0.0001) and short survival (P less than 0.0001). Other characteristics associated with a poor prognosis included hepatomegaly, anemia, and a high percentage of peripheral blast cells at time of diagnosis. When studied in a multivariate analysis, cigarette smoking remained the strongest prognostic factor for both occurrence of blast crisis (P = 0.0003) and overall survival (P = 0.0001). Other poor prognosis factors found in the multivariate analysis included a high percentage of blasts in the peripheral blood at time of diagnosis and high platelet count. It is possible that cigarette smoke may act as a promoter or cocarcinogen in the transformation of chronic myelogenous leukemia.
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PMID:Influence of cigarette smoking on the presentation and course of chronic myelogenous leukemia. 270 75

Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/DR, CD20, FMC7, CD5, and CD9 antigens, had a significant influence on survival. On the other hand, several clinical and haematological characteristics were identified as being associated with survival: 1) clinical features--presence of lymphadenopathies (P less than .05) and hepatomegaly and/or splenomegaly (P less than .04); 2) haematologic parameters--presence of anaemia and/or thrombopenia (P less than .05), the absolute peripheral blood lymphocyte count (P less than .03), and the presence of hypogammaglobulinemia (P less than .08); 3) biochemical parameters--serum uric acid (P less than .03); and 4) bone marrow histopathological features--biopsy pattern (P less than .04) and the percentage of lymphocytes in bone marrow aspirate (P less than .03). Both the Rai staging and the International Workshop on CLL staging systems were effective in identifying groups of patients with significantly different prognoses (P less than .05). Multivariate regression analysis demonstrated that the combination of three clinicopathologic characteristics (bone marrow histopathologic pattern, absolute peripheral blood lymphocyte count, and the presence or not of hypogammaglobulinaemia) had the strongest predictive relationship with survival time. In summary, our findings show that the clinicobiological and anatomopathologic parameters have much more prognostic relevance than the immunological markers analysed in the present study.
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PMID:B-cell chronic lymphocytic leukaemia: prognostic value of the immunophenotype and the clinico-haematological features. 270 40


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