Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Caroli's disease is a rare congenital disorder and occasional cases have been reported from Japan and other parts of Asia. It comprises of congenital dilation of the lower (segmental) intrahepatic bile duct. Cholangitis liver, cirrhosis and cholangiocarcinoma are its potential complication. A case of caroli's disease in an 8-years-old boy with bilobar involvement of liver, (specially affecting right superior lobe) presenting with intermittent abdominal pain, fever and hepatosplenomegaly is reported here.
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PMID:Caroli's disease. 1656 20

Cholestasis is a common feature of several malignant diseases, including pancreatic, hepatic, gallbladder, and ampullary carcinomas. It is usually secondary to main bile duct obstruction or widespread hepatic metastasis, but it can also be a paraneoplastic syndrome of other underlying malignancies. Stauffer's syndrome is a rare paraneoplastic manifestation of renal cell carcinoma (RCC) that is characterized by elevated alkaline phosphatase, erythrocyte sedimentation rate, alpha-2-globulin, and gamma-glutamyl transferase, thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly, in the absence of hepatic metastasis and jaundice. A rare variant of this syndrome with jaundice has recently been described in 3 cases in the literature. We report a patient who presented with abdominal pain and cholestatic jaundice in whom RCC was incidentally found during initial workup. Jaundice and liver dysfunction resolved completely after surgical resection of the tumor. This case illustrates the protean manifestations of RCC, and the importance of considering Stauffer's syndrome and its variant in the differential diagnosis of anicteric and icteric cholestasis, which may allow early recognition and treatment of an underlying malignancy.
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PMID:Stauffer's syndrome variant with cholestatic jaundice: a case report. 1680 61

We describe the case of a 59-year-old Caucasian male who presented to the outpatient clinic with intractable hiccups, upper abdominal pain, repeated bouts of vomiting, and stiff neck. Physical examination revealed a cachectic male with pallor, with enlarged left supraclavicular Virchow's lymph node and hepatosplenomegaly. Histologic examination of the excised lymph node revealed simultaneous presence of 2 malignant processes, nodular sclerosing classical Hodgkin's lymphoma and metastatic adenocarcinoma. Subsequent investigations of the patient, revealed the presence of gastric adenocarcinoma. Although factors governing the coexistence and the possible order of appearance of the 2 pathologies in the present case remain unknown, attempts are made to elucidate the pathogenetic mechanisms that led to their existence.
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PMID:Collision tumor-concurrent involvement of Virchow's lymph node by Hodgkin's disease and metastatic gastric adenocarcinoma. A Troisier's sign and more? 1745 51

We describe the first patient with hereditary spherocytosis (HS) known to have developed splenic infarction following infectious mononucleosis (IM). An 18-year-old Japanese man was referred to our hospital in November 2004 because of continuous fever and icterus. He had undergone cholecystectomy at the age of 14 years. On patient admission in November 2004, a physical examination showed marked hepatosplenomegaly, icterus, and jaundice. He had a white blood cell count of 14.9 x 10(9)/L with 9.5% atypical lymphocytes, a red blood cell count of 2.93 x 10(12)/L, and a hemoglobin concentration of 7.8 g/dL. Microspherocytes were observed in the patient's peripheral blood smear, and immunoglobulin M antibody to Epstein-Barr virus (EBV) viral capsid antigen was detected. The patient's diagnosis was HS with IM. On day 4 of admission, the patient complained of severe abdominal pain. Abdominal computed tomography scanning revealed findings of splenic infarction. Two months after the occurrence of splenic infarction, a splenectomy was performed. A pathohistologic examination of the resected spleen revealed no evidence of thrombosis or arterial occlusion. We assume that the cause of splenic infarction was insufficient blood flow to oxygenate the entire spleen during the acute enlargement of the spleen.
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PMID:Splenic infarction after Epstein-Barr virus infection in a patient with hereditary spherocytosis. 1756 11

We present a case of Budd-Chiari syndrome (BCS) having two risk factors, Behcet's disease (BD) and oral contraceptive (OC) usage. A 33-year-old woman with BD was admitted to the Emergency Unit with nausea, vomiting, abdominal pain, abdominal distention, and confusion started 12 days ago before admission. Since the patient was in a shock state, she was taken to the Intensive Care Unit (ICU) with the suspicion of abdomen-originated sepsis. Abdominal ultrasound showed massive hepatosplenomegaly and moderate ascites. Abdominal MRI revealed an inferior vena cava (IVC) obstruction starting above the renal veins and diffuse thrombosis of the right and medial hepatic veins. An extensive thrombosis of the IVC and the hepatic veins (BCS) which led to shock was diagnosed. In addition to BD, the unnotified OC usage for a year by the patient without her doctor's knowledge was recognized as possible precipitating factor of BCS. Pulse methylprenisolone was started for three consecutive days to treat active BD-induced vasculitis. IVC digital subtraction angiography (DSA) showed occlusion of the IVC below the hepatic veins with extensive collateral circulation originating at the occlusion level suggesting that obliteration had a subacute or chronic course. Since intralesional thrombolytic therapy failed, the patient was transferred to a liver transplantation center. While waiting for an appropriate donor, the patient died due to hepatic failure. Since BCS is mortal and deemed multi-factorial, every patient with a thrombotic risk factor such as BD should be questioned for other possible causes of thrombosis.
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PMID:A case of Budd-Chiari syndrome with Behcet's disease and oral contraceptive usage. 1757 62

A 14-year-old girl was diagnosed with familial Mediterranean fever (FMF) with homozygous for M694V mutation of the MEFV gene and was started on colchicine therapy 4 years before admission to our hospital. She was uncompliant to therapy and was admitted to a local hospital with complaining of fever, malaise, abdominal pain and artralgia lasting for 2 months. Multiple hypoechogenic mass lesions were detected on liver and kidneys with ultrasonography (US) and diagnosed to be hematomas by laparoscopic examination. She was referred to our hospital because of development of convulsions. On physical examination her blood pressure was 140/90 mmHg and body temperature was 39 degrees C. She was pale and extremely cachectic, with atrophic muscles of the extremities. She had diffuse abdominal tenderness and hepatosplenomegaly. Laboratory investigations revealed a hemoglobin of 9.8 g/dl, white blood cell count 9,900/mm3, platelets 213,000/mm3, erythrocyte sedimentation rate (ESR) 112 mm/h, C- reactive protein (CRP) 78 mg/L (normal < 2 mg/L) and fibrinogen 500 mg/dl. Electrolytes, renal and hepatic functions and urinalysis were normal. Examinations of peripheric blood smear and bone marrow aspiration were normal. X-rays of bones and chest showed no pathological finding. Protrombine, partial thromboplastine and bleeding times were normal. Bacterial cultures of blood, urine and stool grew no organisms. Serological tests for hepatitis B and C, cytomegalovirus, salmonella and brucella were negative.
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PMID:Multiple visceral hematomas in a child with familial Mediterranean fever: polyarteritis nodosa. 1818 27

Pyogenic liver abscess (PLA) is a process with significant morbidity and mortality and is a rare complication in an aisled way in patients with autosomal dominant polycystic kidney disease (ADPKD). In addition to hepatic cyst infection, intracystic hemorrhage is another complication seen in ADPKD patients; however, the liver parenchyma itself remains normal. A PLA located in normal liver tissue in these kinds of patients has not been previously reported. Fusobacterium nucleatum is an anaerobic bacterium with rare involvement other than in periodontal infections. A 58-year-old Caucasian male, who was on hemodialysis treatment from July 2004 due to end-stage renal disease secondary to ADPKD, was admitted with fever, rigor, chills, weakness, and abdominal pain of 10 days duration. During that time, ciprofloxacin 500 mg, twice daily, gentamycin 80 mg/48 h, and vancomycin 1 g/week, were prescribed, but treatment was interrupted by hospitalization. Physical examination on admission revealed that the patient had a fever of 39.8 degrees C, pallor, chills, right upper quadrant abdominal pain, and hepatosplenomegaly. Abdominal ultrasound revealed a 5.3 cm diameter collection with irregular configuration located in the caudate lobe. Abdominal computed tomography (CT) showed a large multiloculated hepatic collection. The PLA was managed with antibiotics (metronidazole) and continuous catheter drainage (8Fr drainage catheters [Abocath-T, Abbott, Sligo, Ireland]) into the abscess. Fluid culture was positive for F. nucleatum. Complete remission was obtained after 12 days without complications. We describe a PLA by F. nucleatum, in a very rare location in an ADPKD patient undergoing hemodialysis without complicated cysts, managed with antibiotics and percutaneous drainage with satisfactory resolution.
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PMID:A case report of a pyogenic liver abscess caused by Fusobacterium nucleatum in a patient with autosomal dominant polycystic kidney disease undergoing hemodialysis. 1825 20

We report a case of a 30-year-old man presenting with abdominal pain, fever, homodynamic instability, hepatosplenomegaly, acute renal failure, cervical lymph nodes, anaemia and thrombocytopenia. The patient was treated with empiric antibiotics, high dose corticosteroids, gammaglobulins, noradrenalin and diary intermittent haemodialysis, with an excellent response. The renal biopsy showed a thrombotic microangiopathy, the lymph node biopsy showed a Castleman s disease. Castleman s disease (also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia) is a clinicopathological entity of unknown aetiology. A number of renal alterations have been described in association with the Castleman s disease.
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PMID:[Acute renal failure due to thrombotic microangiopathy associated with Castleman's disease]. 1827 98

Hyperimmunoglobulin D and periodic fever syndrome (HIDS) is an autosomal recessive auto-inflammatory disorder characterized by recurrent febrile attacks with lymphadenopathy, abdominal distress, skin eruptions and joint involvement. We discuss the case of a 15-year-old Japanese girl who had presented with periodic fever, hepatosplenomegaly and intractable diarrhea from seven weeks of age. At first, undifferentiated autoimmune disorder was suspected, and she was treated with prednisolone and, in turn, with immunosuppressants such as cyclosporine, methotrexate, cyclophosphamide and rituximab or with plasma exchange. However, these trials failed to relieve her symptoms, and so she was transferred to our hospital when she was 15 years old. Her parents and elder brother had no history of recurrent fever, prolonged abdominal pain or diarrhea of unknown origin. The patient had extremely elevated levels of mevalonic aciduria and had homozygosity as a novel mutation in the MVK gene (G326R). Finally, HIDS was diagnosed. She was treated with simvastatin, which resulted in a moderate decrease of the urinary mevalonic acid concentration and good clinical course. This is the first case in which homozygosity for the mutation of the MVK gene has been reported in an Asian patient, and indicated a need for differentiation.
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PMID:Hyper-IgD syndrome with novel mutation in a Japanese girl. 1894 11

Disseminated histoplasmosis is an AIDS-defining illness which usually involves the liver and gastrointestinal tract, most commonly the small bowel. Abdominal pain, diarrhea, GI bleeding, hepatosplenomegaly and small bowel obstruction are well described presentations. Still gastrointestinal histoplasmosis often results in either vague symptomatology or no symptoms. Pancreaticobiliary disease related to disseminated histoplasmosis is not well characterized. We report the case of a young female with advanced HIV infection and biliary obstruction and a periampullary duodenal ulcer due to disseminated histoplasmosis.
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PMID:Disseminated histoplasmosis presenting with biliary obstruction and duodenal ulcer. 1963 48


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