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)

Eighty-eight patients with Hemoglobin (Hb) H diagnosed in our hospital in the past decade were reviewed. Among them, 37 were males and 51 were females, and their age ranged from 10 to 77 years. In physical examination, 43.3% of them showed to have jaundice, 47% had splenomegaly, 14% had hepatomegaly and 19.3% had gall stone. Hb electrophoresis revealed the presence of Hb H (1.4-40%), Hb F (23/88, range: 0.5-7.5%) and decreased Hb A2 (mean = 1.23 +/- 1.2%). The study of iron status showed increased serum ferritin concentration (mean = 421.4 +/- 343.7 ug/dl) and transferrin saturation ratio (53.9% +/- 20.5%). Hemosiderosis was found in three out of four patients received liver biopsy. Although most patients did not receive blood transfusion frequently, iron overload was not uncommon in the patients with Hb H disease. Further study would be needed to explore the true relationship between iron overload and Hb H disease.
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PMID:Hemoglobin H disease--ten years' experience. 216 37

Patients with newly diagnosed prostatic cancer should be investigated with regard to the presence or absence of distant metastases by: (1) Clinical history especially of weight loss, recent pain, or analgesics intake. (2) Physical examination, looking especially for hepatic enlargement, peripheral lymph nodes, local bone tenderness. (3) Performance status. (4) Hemoglobin, creatinine, PSA and/or PAP, alkaline phosphatases, liver tests, testosterone. (5) Bone scan with X-ray of doubtful hot spots. (6) Chest X-ray. (7) Ultrasound scans (liver, kidney, lymph nodes) or CT scan may be indicated if abnormal blood parameters or in specific situations. (8) Other investigations are only indicated in special circumstances. Follow-up should include: (1), (2), (3), (4) every 3 months. For patients in clinical trials, depending on the end point, bone scan should be repeated every 6 months or possibly depending on the prognostic group (good: every 12 months; bad: 3 to 6 months). For routine clinical management, it could be repeated only when markers (PAP, PSA, alkaline phosphatase) show significant (25-50%) increase and provided the result will influence treatment. Other investigations should only be repeated or performed if abnormal at the start of if clinical data require them.
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PMID:The staging of M+ disease. 221 62

Patients with newly diagnosed prostatic cancer should be investigated with regard to the presence or absence of distant metastases by (1) Taking a history especially of weight loss and recent onset backache (2) Examining them, looking especially for hepatic enlargement or peripheral lymph nodes (3) Performance status (4) Hemoglobin, Bilirubin, Liver enzymes, Alkaline and Acid phosphatase (5) Chest Xray. (6) Bone scan with specific Xrays directed at hot spots. (7) Ultrasound scan of liver if liver function tests are abnormal. Ultrasound scan of lymph nodes and kidneys is optional. (8) Any other tests indicated in special circumstances. Follow-up, 3-monthly as a rule, should include (1) The presence of pain and analgesic requirements (2) Weight (3) Performance status (4) Hemoglobin, Alkaline phosphatase, Acid phosphatase (5) Chest Xray, three monthly if abnormal. Annually otherwise. (6) Bone scan with Xray of new hot spots, 6-monthly. If there is doubt about the presence of a new hot spot, repeat the bone scan and Xray at 3 months.
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PMID:The staging of M1 disease: the role of bone scan, Xray and other imaging techniques. 329 62

Twenty-one patients of autoimmune hemolytic anemia (AIHA), aged 2 months to 57 years were analyzed. The common presenting feature was pallor (89%), fever (38%), Jaundice (43%) and hepatomegaly and splenomegaly was seen in 76% and 81% respectively. Fifteen cases were of idiopathic etiology and in 6 cases the etiology could be identified as systemic lupus erythematosus, systemic sclerosis, pregnancy, maternal AIHA, typhoid fever and myelodysplastic syndrome (one each). Hemoglobin level ranged between 1.9 to 11.7 gm/dl (mean 6.8 gm/dl) and reticulocyte counts between 6% to 42% (mean (20.2%). Four patients had thrombocytopenia. Direct antiglobulin test (DAT) was positive in 19 and indirect antiglobulin test (IAT) in 7 cases. There was no correlation between DAT positivity and severity of anemia. All patients had warm antibodies of IgG type. Ten of fourteen patients responded to steroid therapy. Patients with secondary AIHA had a significantly poorer prognosis compared to the idiopathic group.
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PMID:Clinico-hematological spectrum of auto-immune hemolytic anemia: an Indian experience. 1099 63

Hairy cell leukaemia variant is a very rare chronic lymphoproliferative disorder and is closely related to hairy cell leukemia. We hereby describe a case of hairy cell leukaemia variant for the first time in Saudi Arabia. An elderly Saudi man presented with pallor, massive splenomegaly, and moderate hepatomegaly. Hemoglobin was 7.7 g/dl, Platelets were 134 x109/l and white blood count was 140x10 9/l with 97% being abnormal lymphoid cells with cytoplasmic projections. The morphology, cytochemistry, and immunophenotype of the lymphoid cells were classical of hairy cell leukaemia variant. The bone marrow was easily aspirated and findings were consistent with hairy cell leukaemia variant.
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PMID:Hairy cell leukemia-variant. 1125 16

Acute hepatic failure has been reported in the presence of Epstein-Barr virus (EBV) infection. Autoimmune hemolytic anemia may also occur in the course of this infection. We report a rare case of fulminant hepatic failure and autoimmune hemolytic anemia associated with Epstein-Barr virus. A seven-year-old girl was admitted with the complaints of abdominal pain, vomiting and jaundice. She was irritable, confused and had mild hepatomegaly with marked splenomegaly. Serum aminotransferase levels were moderately elevated, while direct and indirect bilirubin levels were markedly elevated. Prothrombin time was prolonged. Hemoglobin was 3.9 g/dl. Anti-HAV IgM, HbsAg, anti-HBc IgM, anti-HCV and anti-CMV IgM were negative, while IgM VCA EBV, IgG VCA EBV and anti-CMV IgG were positive. Serum copper and ceruloplasmin levels were normal. The patient received supportive therapy for hepatic failure. Meanwhile, the cause of the deep anemia was investigated and autoimmune hemolytic anemia was ascertained by means of increased reticulocyte count and positive Coombs test. Corticosteroid therapy was administered. The prognosis was good. Although not reported before, the combination of acute hepatic failure and autoimmune hemolytic anemia may complicate the course of EBV infection. Physicians need to be aware of this association.
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PMID:Fulminant hepatic failure and autoimmune hemolytic anemia associated with Epstein-Barr virus infection. 1221 11

An interpretation of historical, clinical, and laboratory data was made to identify the correlates of and the diversity between cerebral malaria (CM) and severe malarial anemia (SMA) in a setting of low, seasonal, and unstable malaria transmission in eastern Sudan. Hemoglobin (Hb), random blood glucose (RBG), and anti-MSP antibodies were measured. Results showed that SMA and CM were significantly different with regard to age, malaria history, fever duration, convulsions, and hepatosplenomegaly. The MSP Ab response was inversely correlated with the number of previous malaria episodes but not with fever duration in the current attack. The spleen size was significantly inversely correlated with Hb level while hepatomegaly was significantly associated with low RBG. Furthermore, two malaria patients presented with neuropsychiatric upset. Finally, the correlates of SMA and CM fit perfectly with an adopted severity numeric scoring.
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PMID:Severe malaria in an unstable setting: clinical and laboratory correlates of cerebral malaria and severe malarial anemia and a paradigm for a simplified severity scoring. 1900 25

Gaucher disease (GD) is a lysosomal storage disorder characterized by anemia and thrombocytopenia, hepatosplenomegaly, and skeletal involvement. The management of Gaucher disease was improved by the development of enzyme replacement therapy (ERT). However, the bone response to ERT is generally slower compared to other clinical manifestations. Some have recommended the early use of ERT to prevent the development of severe skeletal complications. Because we have access to over 30 untreated patients in Ontario, we questioned the extent to which complications progress in severity over a long period of time. We examined retrospectively the natural history of GD and the extent of skeletal manifestations in 22 untreated type 1 GD adult patients (mean age, 49+/-3.3; range, 20-81 years). The patients were followed for a median of 9.5 years (range, 3-16 years). Hemoglobin (Hb) concentration did not significantly change over time (mean baseline concentration of 12.8+/-0.27 g/dL vs. mean recent concentration of 12.6+/-0.37 g/dL, p=0.65). Mean platelet count also remained relatively stable over time (mean baseline count of 138+/-13x10(9)/L vs. mean recent count of 138.5+/-18x10(9)/L, p=0.98). Mean ferritin and ACE concentrations were elevated and were stable over time. Liver volumes decreased over time (mean baseline liver volume of 1.2xnormal (N) vs. mean recent volume of 1.06xN, p=0.27) and 6 of 22 (27%) patients had moderate hepatomegaly (liver volume, 1.25-2.5xN). Spleen volumes remained stable over time (mean baseline spleen volume of 6.6xN vs. mean recent volume of 5.2xN, p=0.5). None of the changes was statistically significant. Four of 20 (20%) patients had moderate splenomegaly (spleen volume, 5-15xN), 2 of 20 (10%) had marked splenomegaly (spleen volume, >or=15xN), and 2 of 22 (9%) had had splenectomy. The most common skeletal manifestations were infiltration of the bone marrow in 16 of 22 (73%) patients followed by osteopenia in 15 of 22 (68%), Erlenmeyer flask deformity in 13 of 22 (59%), and infarctions in 6 of 22 (27%) patients. We observed that bone disease remained relatively stable over time in most patients, although three patients developed new infarcts over time, one developed an avascular necrosis (AVN), and four had an increase in the degree of osteopenia. Although GD and its skeletal complications progress in severity in some patients, our results suggest that GD complications, including bony disease, may stabilize over time. Therefore, early use of ERT may not be necessary in all type 1 GD patients.
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PMID:The clinical course of untreated Gaucher disease in 22 patients over 10 years: hematological and skeletal manifestations. 1979 65

We report the case of a 14 year-old male from Lima. He is a student with a history of bronchial asthma since age 4 receives conditional salbutamol, corticosteroids used for asthma attacks (a crisis in 2010, 1 month ago) Refuses surgery or transfusions. He presented with a two weeks for abdominal pain, nausea, fever, and jaundice. Epigastric pain is colicky and radiated back to righ upper quadrant, refers in addition to nausea and fever, for ten days notice jaundice of skin and sclera. On examen he was lucid, with jaundice of skin and mucous membranes. There was no palpable lymph nodes, abdomen with bowel sounds, soft, depressible, liver span of 15cm, positive Murphy, no peritonitis. The laboratory findings showed hemoglobin 13gr, MCV 90, platelets 461.000/mm3, WBC 4320/mm, lymphocytes 1700 (39%). total bilirubin: 8.8, B Direct: 7.6, ALT (alanine aminotransferase): 3016, AST (aspartate aminotransferase): 984, alkaline phosphatase: 250, albumin: 3.34gr%, globulin: 2.8, amylase: 589 (high serum amylase), TP: 17, INR: 1.6, VHA IgM positive. 89 mg glucose, urea 19 mg%, creatinine 0.5 mg Hemoglobin 13gr, MCV 90 Platelet 461000/mm3, WBC 4320/mm, Lymphocytes 1700 (39%). The nuclear magnetic resonance showed hepatomegaly associated with thickening of gallbladder wall without stones up to 11mm inside. No bile duct dilatation, bile duct 4mm, pancreas increased prevalence of body size. Mild splenomegaly and free fluid in the space of Morrison and right flank. Abdominal ultrasound revealed a gallbladder wall thickness (11mm), without stones in his light. Pancreas to increase volume with peripancreatic fluid free perivesicular with a volume of 430 cc. Findings consistent with acute acalculous cholecystitis and acute pancreatitis. CT-scan showed enlarged pancreas with predominance of body and tail with peripancreatic edema; the gallbladder was thickening. We report this case because the extrahepatic manifestations of viral hepatitis A infection are uncommon, specially the associated with acute acalculous cholecystitis and acute pancreatitis simultaneous.
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PMID:[Acute pancreatitis and acalculous cholecystitis associated with viral hepatitis A]. 2183 59

Dengue usually presents itself with subclinical or mild infection to full blown dengue fever (DF) to dengue hemorrhagic fever (DHF) and dengue shock syndrome (DSS). In Kolkata, dengue started in 1824 followed by five epidemics that occurred in 1836, 1906, 1911, 1923 and 2005. The aim of this investigation is to study the clinicohematological correlation of all patients with respect to their gender that were admitted to "Kali Pada Chowdhury Medical College and Hospital" during 2012 epidemic. Amongst a total of 1237 dengue patients (either dengue Nonstructural protein1 antigen or dengue Immunoglobulin M positive) that were admitted to the hospital, 11 patients died within 48 hours of admission; hence they have been excluded from the study. DHF patients were divided into males and females. During admission, proper history, physical examinations with necessary hematological investigations were performed and repeated again after 24-48 hours. After collection of all the reports, correlations of the collected data were carried out. 170 and 1056 patients were diagnosed with DF and DHF respectively; significant symptoms and signs were headache, backache/myalgia, nausea/vomiting, loose motion and anorexia hepatomegaly. Hemoglobin level was low in females, leucopenia observed in 79.52% patients and thrombocytopenia seen in 57.58% and 86.13% patients during and 24-48 hours after admission respectively. 96 and 97 DHF patients showed evidences of ascites and plural effusion respectively. In 2012 epidemic, 86.13% patients suffered from DHF, headache, backache, nausea/vomiting, loose motion and anorexia were predominant symptoms. Significant number of patients had leucopenia; only few showed evidence of plasma leakage.
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PMID:Clinicohematological profiles of hospitalized patients with dengue in kolkata in 2012 epidemic, west bengal. 2524 47


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