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)

Sickle cell intrahepatic cholestasis (SCIC) is a rare complication seen in sickle cell patients who present with sudden onset of RUQ pain, progressive hepatomegaly, mild elevation of transaminases, coagulopathy, and extreme hyperbilirubinemia. Early recognition of this entity is essential to avoid life-threatening complications. Diagnosis can be challenging given the overlap in clinical presentation with other conditions affecting the hepatobiliary biliary system in sickle cell anemia such as hepatitis, cholecystitis, and hepatic crisis. Treatment is currently limited to exchange transfusion. The authors present two patients with SCIC and cholelithiasis; the clinical picture of one is complicated by choledocholithiasis.
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PMID:Sickle cell intrahepatic cholestasis with cholelithiasis. 1665 Dec 37

Budd-Chiari syndrome is a spectrum of disease states, including anatomic abnormalities and hypercoagulable disorders, resulting in hepatic venous outflow occlusion. Clinical manifestations observed in the majority of patients include hepatomegaly, right upper quadrant pain, and abdominal ascites. This article outlines the approach to clinical diagnosis and supportive medical therapy in patients who have BCS and reviews the clinical data supporting surgical shunting and liver transplantation as viable treatment options in this patient population.
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PMID:Budd-Chiari syndrome. 1697 Dec 61

Malignant hepatic epithelioid hemangioendothelioma (HEH) is a rare malignant tumor of vascular origin with unknown etiology and a variable natural course. The authors present a comprehensive review of the literature on HEH with a focus on clinical outcome after different therapeutic strategies. All published series on patients with HEH (n = 434 patients) were analyzed from the first description in 1984 to the current literature. The reviewed parameters included demographic data, clinical manifestations, therapeutic modalities, and clinical outcome. The mean age of patients with HEH was 41.7 years, and the male-to-female ratio was 2:3. The most common clinical manifestations were right upper quadrant pain, hepatomegaly, and weight loss. Most patients presented with multifocal tumor that involved both lobes of the liver. Lung, peritoneum, lymph nodes, and bone were the most common sites of extrahepatic involvement at the time of diagnosis. The most common management has been liver transplantation (LTx) (44.8% of patients), followed by no treatment (24.8% of patients), chemotherapy or radiotherapy (21% of patients), and liver resection (LRx) (9.4% of patients). The 1-year and 5-year patient survival rates were 96% and 54.5%, respectively, after LTx; 39.3% and 4.5%, respectively, after no treatment, 73.3% and 30%, respectively, after chemotherapy or radiotherapy; and 100% and 75%, respectively, after LRx. LRx has been the treatment of choice in patients with resectable HEH. However, LTx has been proposed as the treatment of choice because of the hepatic multicentricity of HEH. In addition, LTx is an acceptable option for patients who have HEH with extrahepatic manifestation. Highly selected patients may be able to undergo living-donor LTx, preserving the donor pool. The role of different adjuvant therapies for patients with HEH remains to be determined.
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PMID:Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. 1701 35

Human infection with the Fasciola hepatica liver fluke is a rare cause of biliary obstruction, especially within the United States. Humans can become accidental hosts of this parasite by ingesting contaminated drinking water or plants. Symptoms during the acute phase include fever, right upper quadrant pain, eosinophilia, and hepatomegaly. During the chronic stage of infection, the parasite may remain asymptomatic for many years. This article presents a case of biliary obstruction due to Fasciola hepatica that was diagnosed and treated successfully with endoscopic retrograde cholangiopancreatography.
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PMID:Therapeutic endoscopic retrograde cholangiopancreatography for the treatment of Fasciola hepatica presenting as biliary obstruction. 1705 82

We report a case of hepatic inflammatory myofibroblastic tumor in a 26-year-old African American man who presented with right upper quadrant pain, weight loss, and fatigue during the previous year. Hepatomegaly was found on physical examination. Laboratory findings were significant for mild normocytic, normochromic anemia and elevated erythrocyte sedimentation rate. Imaging studies showed 2 contiguous masses suspicious for malignancy. A left partial hepatectomy was performed; the preoperative differential diagnosis was for angiosarcoma and hepatocellular carcinoma. The resected liver specimen showed 2 contiguous, firm, tan-white nodules that microscopically represented a proliferation of spindled myofibroblast cells set in an inflammatory and collagenized background. The spindle cells were strongly reactive for smooth muscle actin but negative for ALK-1. The morphologic and immunophenotypic findings, coupled with the clinical presentation, were consistent with an inflammatory myofibroblastic tumor of the liver.
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PMID:Inflammatory myofibroblastic tumor of the liver. 1709 Feb

The case of a 36-year-old male professional bodybuilder is reported. He presented to the accident and emergency department with right upper quadrant pain. This was on the background of a 15-year history of anabolic steroid and growth hormone misuse. Examination revealed mild hepatomegaly and a random blood sugar of 30.2 mmol/l. There was no evidence of ketonuria or acidosis. Biochemical evidence of hepatitis was found, and the patient was in acute renal failure. He was given a sliding scale of insulin and an intravenous infusion of crystalloid. The hepatitis and hyperglycaemia settled with conservative treatment. It is believed that this is the first reported case of frank diabetes precipitated by supraphysiological recreational growth hormone misuse.
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PMID:Strong diabetes. 1732 62

Pyogenic liver abscess in patients with Crohn's disease is not common, but the mortality has been reported to be high if diagnosis or treatment is delayed. Herein, we report the case of a 42-year-old man in whom multi-locular liver abscess was encountered in the right lobe of the liver as a complication of Crohn's disease. On his admission to the emergency unit, he had right upper quadrant pain, fever of 39 degrees C and hepatomegaly. Ultrasonography and abdominal computerized tomography demonstrated multi-locular pyogenic liver abscess in the right lobe of the liver. Percutaneous drainage failed because of the density of the pus; laparoscopic exploration was subsequently performed and 1500 ml pus was successfully drained. The patient became clinically stable without fever and with a decreasing white blood cell count on the third postoperative day.
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PMID:Laparoscopic treatment of pyogenic liver abscess complicating Crohn's disease: a case report. 1745 Apr 99

In its invasive form, the trophozoite is responsible for clinical syndromes, ranging from classical dysentery to extraintestinal disease with emphasis on hepatic amebiasis. Abdominal pain, tenderness and diarrhea of watery stool, sometimes with blood, are the predominant symptoms of amebic colitis. Besides the microscopic identification of Entamoeba histolytica, diagnosis should be based on the detection of specific antigens in the stool or PCR associated with the occult blood in the stool. Amebic dysentery is treated with metronidazole, followed by a luminal amebicide. The trophozoite reaches the liver causing hepatic amebiasis. Right upper quadrant pain, fever and hepatomegaly are the predominant symptoms. The diagnosis is made by the finding of E. histolytica in the hepatic fluid, or in the necrotic material at the edge of the lesion in a minority of patients, and by detection of antigens or DNA. Ultrasonography is the initial imaging procedure indicated. The local perforation of hepatic lesion leads to important and serious complications.
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PMID:Invasive amebiasis: an update on diagnosis and management. 1791 22

Fascioliasis is highly endemic in the Andean region of South America. Newer serological assays have improved our ability to diagnose acute fascioliasis. The diagnosis was established by Fasciola hepatica serology (Fas2-ELISA or Western blot) in 10 patients. Identifiable exposure included ingestion of watercress (N = 8), alfalfa juice (N = 5), and lettuce (N = 1). Computed tomography of the abdomen showed hepatomegaly (N = 9), track-like hypodense lesions with subcapsular location (N = 8), and subcapsular hematoma (N = 2). Radiologic sequelae included cyst calcifications detectable at least 3 years after treatment. Stool examinations were negative for F. hepatica eggs; serology was positive (Arc II [N = 2], Fas2-ELISA [N = 6], Western blot [N = 2]). The syndrome of eosinophilia, fever, and right upper quadrant pain, elevated transaminases without jaundice, hypodense liver lesions on CT, and an appropriate exposure history suggests acute fascioliasis. Fascioliasis is specifically treatable with a single dose of triclabendazole.
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PMID:Natural history, clinicoradiologic correlates, and response to triclabendazole in acute massive fascioliasis. 1825 19

A seven-year-old male patient presented to Tikur Anbessa Hospital with right upper quadrant pain of nine months and right upper quadrant swelling of six months duration. He was having loss of appetite and significant weight loss. Physical examination revealed an enlarged liver. He was worked up with laboratory and radiologic investigations. Ultrasound (US) was done and findings were suggestive of the diagnosis and Computed Tomography (CT) scan was done, and was also diagnostic and confirmed by the final histological diagnosis. We present and discuss a case with an uncommon hepatic neoplasm occurring at unusual age.
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PMID:A case of hepatoblastoma; uncommon hepatic neoplasm occurring at unusual age. 1833 Mar 33


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