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)

Forty-seven patients who underwent renal transplants were followed clinically and were examined for serologic or virologic evidence of cytomegalovirus (CMV) infection. There were 18 cases of primary infection and ten cases of secondary infection. These findings were based on whether the patient was seronegative or seropositive prior to transplantation. Thirteen patients with primary infection and only one patient with secondary infection had two or more of the following manifestations that are temporally associated with laboratory evidence of infection: fever, leukopenia, atypical lymphocytes, lymphocytosis, hepatosplenomegaly, myalgia, arthralgia, and pneumonitis. Five patients with primary infections, one of whom died with disseminated disease, were recognized by attending physicians as having CMV disease. Since primary infection is though to be largely due to virus transmitted by the kidney of a seropositive donor, it may be possible to prevent symptomatic primary infection by using only seronegative donors for seronegative recipients.
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PMID:Primary and secondary cytomegalovirus infection. 19 47

Brucellosis has always been an unusual disease in children and, concomitant with the control of the disease in domestic animals, reports have become sparse. The pediatrician, therefore, may not be aware of the protean clinical manifestations of childhood brucellosis. In 1973, nine cases occurred during a three-month period in El Paso, Texas. All cases were marked by spiking fevers and lethargy of four days to four weeks in duration. Tender hepatomegaly or splenomegaly was striking in seven patients. Other characteristics included epistaxis, arthralgia, myalgia, and weight loss. Leukopenia and leukemoid reaction were found in five patients. All of the patients tested had elevated liver enzymes. Febrile agglutinins were invaluable in screening for an early clue to diagnosis. When Brucella abortus antigen agglutinated serum from patients with a positive screen in dilutions greater than 1:320, a presumptive diagnosis of brucellosis was made. Brucella was isolated from the blood or bone marrow in seven patients and the time of incubation proved crucial for successful recovery. Bacterial blood cultures are usually discarded at ten days of age, as were cultures from the only two patients from whom the organism was not recovered. All of the cultures incubated for 12 to 15 days grew B. melitensis, an unusual causative species in the United States. However, several patients admitted eating cheese from the State of Chihuahua, Mexico, made from unpasteurized goat's milk, the presumed source of the infection. Within one to three days, all patients responded dramatically to antibiotics; tetracycline was given orally for 21 days and streptomycin intramuscularly for 14 days. Pediatricians caring for patients in areas where consumption of unpasteurized milk products is likely would do well to consider brucellosis in a child with obscure fever or toxic hepatosplenomegaly.
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PMID:Brucellosis in childhood. 80 83

Fifty-seven (36%) of a cohort of 157 children with brucellosis from Saudi Arabia had arthritis. Most gave a history of contact with farm animals or drinking unpasteurized milk. Associated features included pyrexia, arthralgia, hepatosplenomegaly, and lymphadenopathy. A subacute presentation with peripheral oligoarthritis predominantly affecting hips or knees was common. Specific chemotherapy resulted in rapid defervescence followed by slower resolution of the arthritis. Children with osteoarticular brucellosis had a higher relapse rate and a longer hospital stay. Supervised combination chemotherapy for at least 6 weeks was effective in preventing relapse. A brucellar etiology should be considered in any child from an endemic area who has osteoarticular manifestations.
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PMID:Osteoarticular complications of childhood brucellosis: a study of 57 cases in Saudi Arabia. 145 54

We present a case of acute monocytic leukemia presenting with extramedullary disease clinically resembling lymphoma. A 36 year-old man presented with arthralgia and was found to have skin eruption, nasopharyngeal mass, hepatosplenomegaly and superficial lymphadenopathy. The biopsies of those lesions as well as bone marrow revealed infiltration of CD45 positive large atypical cells and were interpreted as malignant lymphoma. At that time blood film showed leuko-erythroblastic picture, but no atypical cells were noted. He was treated with CHOP followed by VEPA chemotherapy and achieved partial remission. Four months later headache and double vision occurred with the same atypical cell in the cerebrospinal fluid. The cells were identified as monoblasts by cytochemistry and immunophenotype. The diagnosis of aleukemic acute monocytic leukemia was made and its CNS involvement was successfully treated with chemotherapy with DHAP and intrathecal MTX. However, two months later, blasts finally appeared in the peripheral blood and the patient died of multiorgan failure eight months after presentation. The blasts were positive for T cell markers (CD2, 4, 8) and NK marker (NKH-1) as well as monocytic markers. This finding, together with those by other investigators, may indicate the association between these immunophenotypes and extramedullary manifestations of acute monocytic leukemia.
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PMID:[Extramedullary diseases as presenting features of aleukemic acute monocytic leukemia]. 146 83

One-hundred-and-fifty-seven children admitted with brucellosis at Abha, Saudi Arabia, were studied prospectively. Ninety-two per cent gave a history of animal contact, usually with sheep or goats, or ingesting raw milk, milk products, or raw liver. Three-quarters of the patients had an acute or subacute presentation with diverse symptomatology: fever (100 per cent), malaise (91 per cent), anorexia (68 per cent), cough (20 per cent), abdominal symptoms (20 per cent), arthralgia (25 per cent). Hepatomegaly (31 per cent), splenomegaly (55 per cent), and lymphadenopathy (18 per cent) were common findings. Organ complications were rare except for arthritis (36 per cent) which usually presented as a peripheral oligoarthritis involving the hips and knees. All patients had significant agglutination titres; B. melitensis was grown from the blood in 7 of 16 (44 per cent) patients. Haematological variations were common, but non-specific: anaemia (64 per cent), thrombocytopenia (28 per cent), leucopenia (38 per cent), leucocytosis (12 per cent), and elevated erythrocyte sedimentation rate (81 per cent). Varying combinations of rifampicin, co-trimoxazole, tetracycline, and streptomycin resulted in a prompt pyrexial response (mean: 3.8 days), and a slower response in the arthropathy and hepatosplenomegaly. Relapses were related to poor compliance, use of a single drug or a shorter duration of chemotherapy. Brucellosis is a common childhood problem in southwestern Saudi Arabia as in other parts of the country and the Middle East. It should be considered in every child from an endemic area presenting with a febrile illness and a history of animal contact.
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PMID:Childhood brucellosis in southwestern Saudi Arabia: a 5-year experience. 152 11

A 19-year-old boy, who complained of fever and fatigue was hospitalized in November 1986. On physical examination, he had a temperature of 37 degrees C, cervical lymphadenopathy and hepatosplenomegaly. Serum transaminase was elevated moderately, while serum alkaline-phosphatase was elevated severely. Extremely elevated antibody titers to the EBV capsid antigen (IgG: 2560x, IgA: 160x), early antigen (IgG: 1280x, IgA: 160x) and nuclear antigen (160x) were noted. PPD and DNCB skin test were negative. Severe mobilization of Kupfer cells and mild proliferation of pseudoductule were seen in liver biopsied specimen. Cervical lymphnode biopsy showed necrotizing lymphadenitis associated with proliferation of histiocyte. In February 1987 his temperature was elevated to 40 degrees C and he had arthralgia and exanthema. Intravenous Acyclovir (500 mg every 8 hours) and Interferon alpha (6 million u/day) were administered together for 1 month. After that he improved for about a week. In March 1987 he had dyspnea. Arterial blood gas analysis in room air showed a PO2 of 51.8 mmHg, a PCO2 of 28.9 mmHg. A chest radiograph showed thickening of bilateral bronchial walls and obscurity of pulmonary vascular shadows. The effects of transfer factor and Interleukin-2 were unremarkable. High antibody titers to EBV, liver dysfunction and hypo-oxygenemia continued. He died of respiratory and heart failure on 24 October 1987. The most interesting finding of autopsied specimens was stenosis of pulmonary artery associated with interstitial pneumonitis. Hemophagocytosis was seen in liver, spleen and bone marrow.
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PMID:[An autopsied case of chronic active Epstein-Barr virus (EBV) infection with various symptom]. 164 35

One hundred two children, 45 days to 14 years of age, with proven brucellosis were studied to illustrate the epidemiologic, clinical and laboratory findings and to assess the outcome of antimicrobial therapy. The main source of infection was the consumption of raw milk in 80% of the patients. The predominant presenting symptoms and signs were fever, arthralgia, malaise, weight loss, arthritis, hepatosplenomegaly and lymphadenopathy. Brucella melitensis was isolated from 75% of 87 patients. Diverse hematologic and biochemical abnormalities were found. Different durations and combinations of trimethoprim-sulfamethoxazole or tetracycline plus streptomycin or rifampin were used for therapy. Eight-five patients were followed for an average of 14 months. Twelve (85.7%) of 14 patients treated with two-antibiotic combinations for 3 weeks relapsed, as did 5 (8%) of 62 patients treated for at least 6 weeks (P less than 0.001). No relapses occurred in 9 patients treated with trimethoprim-sulfamethoxazole and rifampin for 8 to 12 weeks plus streptomycin for the first 3 weeks. Longer duration and combination of antibiotic therapy seem warranted to improve outcome and to prevent relapses.
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PMID:Childhood brucellosis: a study of 102 cases. 226 41

A 70-year-old man, who had been diagnosed as primary myelofibrosis in 1987 at Tokyo Women's Medical School, was admitted to our hospital because of left hip-joint pain in May 1988. Physical examinations revealed marked hepatosplenomegaly and multiple reddish papules on the skin. The peripheral blood showed marked leukoerythroblastosis and severe anemia with poikilocytosis. Bone marrow aspirations were dry tap. Needle biopsy of iliac crest showed a diffuse fibrosis. Biopsy of the papules showed an extramedullary haematopoiesis. He was treated with low dose Ara-C for seven days. Although hepatosplenomegaly and skin papules were reduced, he died of pneumonia a month later. At autopsy, leukemic cells massively infiltrated into the multiple organs, including bone marrow. The cells were identified with megakaryoblast, as those were positive for factor VIII related antigen. As far as we know, this is the third case of primary myelofibrosis transformed into acute megakaryoblastic leukemia.
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PMID:[Primary myelofibrosis with extramedullary haematopoiesis of the skin transformed into acute megakaryoblastic leukemia]. 232 83

One hundred and twenty-five cases of biopsy proven sarcoidosis have been found during a prospective study since 1972 in Calcutta, Eastern India. The presentation, clinical course and radiological features are considerably different from those seen in the West. Elderly males over 40 years are more prone. Low grade fever, cough, dyspnoea, arthralgia are common symptoms while hepatosplenomegaly, hypercalcaemia, hypercalciuria and hyperglobulinaemia are frequent signs. Nearly 60% are MT negative (up to 100 TU). Serum angiotensin converting enzyme and high lymphocyte count in bronchoalveolar lavage fluid are usual findings in active disease. Chest X-ray usually shows mottled opacities or fibrosis in 60% cases. Clinico-radiological dissociation (i.e. remarkable dissociation between the alarming-looking chest X-ray and scanty physical signs and symptoms in chest) was a very remarkable feature in this series. Treatment with oral steroid or steroid aerosol with oxyphenbutazone and chloroquine give equally good results initially. However, most cases tend to relapse inspite of adequate initial treatment. The pattern of the disease is similar almost all over India with minor regional differences like more erythema nodosum and eye involvement in Chandigarh in the extreme north (which could also have been due to case selection). The pattern from Northern India (Delhi) and Western India is nearly similar to our figures.
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PMID:Sarcoidosis in India: a review of 125 biopsy-proven cases from eastern India. 234 18

Essential mixed cryoglobulinemia (EMC) is a syndrome characterized by cryoglobulinemia and clinical features including purpura, arthralgia, asthenia (Meltzer-Franklin syndrome) without evidence of any systemic disease Liver involvement in the course of EMC is described in 50-84% of patients. It consists of mild silent hepatosplenomegaly and slightly rise of serum amino transferase. Eleven patients with clinical and laboratory findings suggestive for EMC (five type II and six type III) underwent percutaneous liver biopsy to evaluate the degree of liver involvement. Two liver cirrhosis, two chronic active hepatitis, one chronic persistent hepatitis and a case of hepatic steatosis were found. A type III cryoglobulinemia was present in four of the six patients with liver involvement. All the patients were Hbs Ag negative but three of them were Hbs Ab positive. The pathogenesis of liver involvement in the course of EMC is still now uncertain. The authors believe that a previous HBV infection plays no role in the pathogenesis of EMC syndrome. This syndrome must be considered different from mixed cryoglobulinemia secondary to chronic liver disease. They suggest that liver biopsy is mandatory during the course of EMC even when clinical and laboratory data are silent.
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PMID:[Essential mixed cryoglobulinemia with liver involvement: a still open problem]. 238 52


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