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)

Budd-Chiari syndrome is an unusual form of portal hypertension, resulting from partial or total obstruction of the major hepatic veins or of inferior vena cava. This is illustrated by the clinical history of a two year old girl admitted by anemia, hepatomegaly, thrombocytopenia, pleural effusion, ascites and ultrasonographic, scintigraphic and angiographic evidence os suprahepatic veins and inferior vena cava obstruction. Liver biopsy showed signs of chronic congestion and fibrosis. Initial manifestation of illness may be acute or, as in this patient, with an insidious onset of symptoms. Medical therapy gives usually poor results. Several surgical approaches have been tried to alleviate liver congestion, which in this case was intended by a 6 mm wide Goretex ringed tube prosthesis extending from superior mesenteric vein throughout right atrium. Patency of the artificial shunt was proven after several days by Doppler ultrasound but the patient died of pneumonia two months after operation.
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PMID:[Budd-Chiari syndrome]. 184 28

Between January 1981 and December 1990, 41 patients were operated on for hepatic hydatid disease, representing 18% of the overall surgery for liver disease performed in our institution during the same period of time. The mean age of the patients was 39.5 years (range 17-71 years), 24 were females and 17 males. The most common clinical sign was abdominal pain (59%) with 3 patients jaundiced at the time of admission. 2 patients suffered from acute rightsided pneumonia, one of them with a very important productive cough due to bronchial perforation of the hydatid cyst of the liver. One patient was admitted due to anaphylactic shock with collapse. For 14 patients (34%) the discovery of the disease was casual, either by clinical hepatomegaly or by X-ray investigations performed for other reasons. We performed 18 pericystectomies, 4 subtotal pericystectomies and 16 liver resections. Only 3 patients were treated by partial cystectomy with removal of the prominent part of the cyst. Several other surgical procedures were performed concomitantly, including 2 pulmonary lobectomies, 1 cystojejunostomy, and pericystectomies for other hepatic or extrahepatic localizations of hydatid cysts. Rupture of the cyst into the bile duct was diagnosed in 13 patients. One patient presented with a peritoneal rupture of the cyst and 2 patients had a fistula through the diaphragm into the pleural cavity. Our postoperative morbidity is acceptable (10 patients, 25%). A bilioma and a biliary leak were successfully treated by percutaneous drainage, a postoperative hemorrhage required surgical treatment, four patients experienced pulmonary complications and the three others minor wound abscesses.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Surgical treatment of hepatic hydatidosis]. 192 52

Clinical and laboratory manifestations, disease course, outcome, and HLA associations were studied in an inception cohort of 62 subjects with adult Still's disease (ASD) from 5 Canadian universities. Twenty-eight patients (45%) were female and the median age at disease onset was 24 years. In general, the clinical features observed in our patients were identical to those in other published series. However, significantly higher frequencies of sore throat (92%), weight loss (76%), lymphadenopathy (74%), pleuritis (53%), pneumonitis (27%), and abdominal pain (48%) were noted in our patients compared to those in a recent literature review. Liver involvement with hepatomegaly (44%) or abnormal liver function tests (LFTs) (76%) was common and was responsible for the 2 deaths attributed to Still's disease in our series. Severe liver failure always occurred in conjunction with aspirin or NSAID therapy. Therefore, whether or not aspirin or other NSAIDs are used, we recommend close monitoring of LFTs in patients with ASD, especially early in the disease course. Laboratory manifestations were similar to those already reported. Leukocytosis (greater than or equal to 15,000/mm3) was present in 50 patients (81%), a normochromic, normocytic anemia (hemoglobin less than or equal to 10 g/dl) in 42 (68%), and an elevated ESR in all. The mean follow-up of the 62 patients was 70 months (range, 2-163). Twenty-one patients (34%) had a self-limited disease course, 15 (24%) an intermittent course, and 22 (36%) a chronic disease course. Four patients (6%) died, and 2 of these deaths were attributed to Still's disease. For those patients who experienced a recurrence of ASD, the flares were usually of shorter duration and milder in severity than the initial episode. No initiating factor for disease exacerbation was identified in our patients. Although 22 of 62 patients (36%) had a chronic disease course, 52 (90%) were in ARA Functional Class I, and only 4 and 2 patients were in ARA Functional Class II and III, respectively. Patients with Still's disease had higher scores than the controls on the Pain (P less than 0.01) and Physical Disability (P less than 0.05) subscales of Arthritis Impact Measurement Scales health status questionnaire. Joint radiographs performed at the follow-up evaluation disclosed typical carpometacarpal and intercarpal involvement in 16 of 39 patients. In our series, HLA-B17, B18, B35, and DR2 were significantly associated with ASD. Three significant predictors of an unfavorable outcome, either a chronic disease course or a longer time to clinical remission, were identified.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Adult Still's disease: manifestations, disease course, and outcome in 62 patients. 200 77

Four newborns with adenovirus infection are described, and the profile of neonatal adenovirus disease is outlined based on the cases of these newborns and nine previously described. Characteristic historical features included prolonged rupture of membranes, maternal illness, vaginal delivery, and onset of illness within the first 10 days of life. Clinical findings included lethargy, fever or hypothermia, anorexia, apnea, hepatomegaly, bleeding, and progressive pneumonia. Thrombocytopenia, coagulopathy, and hepatitis were typical laboratory manifestations. Illness was severe and generally unremitting; only two survivors have been reported. Pathologic changes were prominent in lung, liver, and brain. Virus isolates, predominantly serotypes 3, 7, 21, and 30 were obtained from multiple sites and organs. Epidemiologic evidence suggests that viral acquisition from the mother, perhaps via the birth canal, is a major mode of transmission. Neonatal adenovirus infection, which is frequently disseminated and generally fatal, should be considered in the differential diagnosis of neonatal sepsis and pneumonia.
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PMID:Neonatal adenovirus infection: four patients and review of the literature. 203 95

Between October, 1985, and February, 1987, 28 (8.7%) cases of scrub and murine typhus were diagnosed among 320 children with greater than or equal to 1 week history of obscure fever. Scrub typhus is a rural disease and characterized by fever, tachypnea and hepatosplenomegaly. Skin rash was rare and eschar was absent. Four patients had pneumonia and two had meningitis. Murine typhus, more an urban disease, was milder and half the patients presented exclusively because of night fever. Slightly enlarged liver and skin rash were the only significant physical signs. Lacking the classical textbook presentations, both rickettsioses often were missed or diagnosed as enteric fever. Recognition is important because patients with either disease respond well to treatment with chloramphenicol or doxycycline.
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PMID:Scrub and murine typhus in children with obscure fever in the tropics. 204 66

As many as 38 children aged 2 to 12 months suffering from acute pneumonia complicated by infectious-toxic shock manifested dilatation of the veins of portal circulation and deposition of a considerable blood volume, which was revealed by ultrasonic examination. The hemodynamic disorders were shown to play the leading part in the genesis of hepatomegaly, followed by hypoxic derangement of the hepatocyte. In infants, who are in a state of infectious-toxic shock, vascular decompensation of the liver is more pronounced and sets in earlier than parenchymatous. Administration of troxevasin (30 mg/kg a day) to the sick children promotes a more rapid elimination of venous dilatation in the system of portal circulation.
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PMID:[Status of the venous part of the portal system in infectious-toxic shock in infants with acute pneumonia]. 207 39

Of 70 consecutive patients with amoebic liver abscess admitted over a 3 year period, 15 (21.4%) had multiple abscesses. This condition, like solitary abscess, was a disease of the 2nd to 5th decade with a male preponderance. Multiple abscesses were more frequently associated with fever, jaundice, upper abdominal pain, pneumonitis and tender hepatomegaly. The left lobe of the liver was always enlarged in patients with multiple abscesses and 86% of patients required drainage of an abscess cavity. The presence of more severe clinical course, jaundice and left lobe hepatomegaly should raise the suspicion of multiple abscesses. After confirmation of the diagnosis by imaging technique, the abscess cavity should be aspirated for quick relief and cure.
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PMID:Clinical profile of multiple amoebic liver abscesses. 207 68

Listeriosis of the newborn is a relatively rare disease, presenting with clinical signs of septicemia. Early onset disease, resembling group B streptococcal septicemia, is already transmitted from the mother to the fetus and is associated with high morbidity and mortality. Late onset septicemia occurs as sporadic or as epidemic disease, usually beyond the fifth day of life. Epidemics can be caused by consumption of contaminated food or by nosocomial infections in neonatal units. Phage typing offers an opportunity to elucidate the route of transmission. During a 7 years period, 5 neonates ware diagnosed to have early onset, 1 newborn to have late onset Listeriosis. They all showed signs of bacterial septicemia with typical changes of white blood cell count, elevated CRP, hepatomegaly, and severe pneumonia. In all patients Listeria monocytogenes could be isolated from blood cultures. Serological tests were negative in all cases. 3 patients died. Nosocomial transmission of Listeria monocytogenes from one infant to another was proven by phage typing.
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PMID:[Listeriosis in newborn infants]. 212 91

Twelve one-day-old chicks were experimentally inoculated with Chlamydia psittaci derived from turkeys. Acute chlamydial septicemic lesions were induced by the inoculation into the air sac and trachea. No lesions were produced by the esophageal injection. Clinically, the affected chicks showed emaciation and mouth breathing, and were inactive while some birds died. Grossly, they had hepatomegaly, splenomegaly and airsacculitis. Histopathologically, fibrinopurulent airsacculitis, pneumonia and bronchitis, multiple fibrinous serositis in the hepatic and splenic capsules, peri- and epicardium, and mesenterium, focal endoarteritis in the aortae, activation of reticuloendothelial cells in the spleen, and hepatic necrosis were noted. Immunohistochemically, chlamydial antigen granules were present in the cytoplasm of epithelial cells of the respiratory system, hepatocytes, macrophages in the air sac, lung, serous membrane, liver, spleen, aortae, reticuloendothelial cells in the spleen, and mesothelial cells in various organs or tissues. Chlamydial multiplication in the cells of the organs or tissues involved was preceded to form the lesions.
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PMID:Pathology of experimental chlamydiosis in chicks. 214 Aug 67

The first twenty-one cases of Paediatric Acquired Immunodeficiency Syndrome (PAIDS) in Trinidad and Tobago were studied. An overwhelming majority of patients were of African descent. Most of the children presented within the first year of life, the average time between presentation and death was 4 1/2 months, and the majority presented with either diarrhoea or pneumonia or failure to thrive, common conditions in the West Indies. Fever lasting longer than two weeks as well as hepatomegaly were clues which led to a definitive diagnosis.
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PMID:Paediatric acquired immunodeficiency syndrome (PAIDS) in Trinidad and Tobago. 226 32


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