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)

A 73-year-old man was admitted to our hospital on April 30, 1990, because of fever persisting for 18 months. Bone marrow puncture and biopsy were performed, because examination on admission revealed an elevated leukocyte count and anemia while his superficial lymph nodes, liver and spleen were not palpable. The results of the bone marrow biopsy revealed evidence of granuloma. Around May 10, the patient developed hepatosplenomegaly and enlargement of left cervical lymph nodes. Based on the results lymph node biopsy, a diagnosis of Hodgkin's disease was made, and CHOP therapy was instituted on May 20. However, the patient developed interstitial pneumonia and died on July 3. This patient's disease was manifested by fever of unknown origin. Bone marrow biopsy revealed granuloma with histiocytes predominating, and the patient subsequently developed lymph node enlargement. His disease was then diagnosed as Hodgkin's disease on the basis of a biopsy. Malignant lymphomas associated with granulomas in the bone marrow, liver or spleen are for the most part found in the advanced stage of the disease. It should be borne in mind, however, that some patients may exhibit granuloma formation in their bone marrow prior to lymph node enlargement or hepatosplenomegaly, as in the present case.
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PMID:[Hodgkin's disease presenting with fever of unknown origin associated with granulomas of the bone marrow]. 143 47

In a young man who had a prolonged fever of unknown origin, hepatosplenomegaly, and progressive pancytopenia, stained smears, blood-agar cultures of bone marrow, and serologic testing for antileishmanial antibodies were negative. Biopsies from liver and bone marrow were uninformative. Visceral leishmaniasis was diagnosed only after splenectomy, when amastigotes were finally cultured from the spleen. The parasite was shown to be an unusual leishmanial parasite, possessing a mixture of intrinsic biochemical and serologic characteristics displayed independently by Leishmania tropica and Leishmania donovani sensu lato, the latter being the usual cause of visceral leishmaniasis. After splenectomy, parasites were also demonstrated in stained bone marrow aspirate smears. Recovery was uneventful after treatment with antimony for 28 days. Visceral leishmaniasis can be a cause of fever of unknown origin and should be considered in its differential diagnosis in endemic areas.
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PMID:Visceral leishmaniasis: a difficult diagnosis and unusual causative agent. 165 58

Wolman's disease is a rare inherited disorder of lipid metabolism in which large amounts of triglycerides and cholesteryl esters accumulate in the visceral organs. The main clinical features of the infantile form of the disease are failure to thrive, vomiting and diarrhoea, hepatosplenomegaly and radiological evidence of calcification of the adrenals. We were able to follow the course of this disease in a female turkish infant. It was first admitted because of a transient swelling within the right angle of mandible, subfebrile temperatures and abdominal distension as well as vomiting at the age of three days. After symptomatic treatment she was discharged home without a specific diagnosis. At the age of 4.5 months she was readmitted with severe hepatosplenomegaly, hypochromic anemia and fever of unknown origin. Calcifications of the adrenals and lymphocytic vacuoles led to the diagnosis of Wolman's disease. Deficiency of acid lipase activity in leucocytes could establish this diagnosis.
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PMID:[Wolman's disease in an infant]. 232 17

A 3-year-old Wisconsin native with the symptoms of chronic fever, hepatosplenomegaly, and inability to walk, was found to have an elevated anti-Brucella titer. Blood and bone marrow cultures grew Brucella melitensis, biotype 3. This infection was likely acquired during a trip to Mexico several months earlier during which the child had ingested raw milk. The patient's father was also discovered to have brucellosis. Both were successfully treated with antibiotic therapy. Although brucellosis has been almost totally eradicated in the United States, in the appropriate setting it should be considered as a possible cause of fever of unknown origin in a child.
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PMID:Brucellosis: an unusual cause of a child's fever of unknown origin. 272 70

Forty-one black patients aged 21 to 75 years with hepatic tuberculosis diagnosed at liver biopsy were studied prospectively. The liver varied in size and consistency and was tender in 44 per cent of patients. Abdominal symptoms, weight loss, pyrexia, hepatomegaly, splenomegaly and anaemia were absent in 54, 39, 37, 5, 68 and 27 per cent of patients respectively. Twenty-two per cent of chest radiographs were normal. Liver function tests were of little diagnostic value and hepatic imaging techniques often gave normal results. Acid-fast bacilli, caseation and coexistent liver disease were detected in 59, 51 and 37 per cent of patients respectively. Since there was no consistent clinical pattern a high index of suspicion is necessary if this disease is to be detected in communities in which tuberculosis is endemic. In patients with unexplained hepatomegaly or hepatosplenomegaly or pyrexia of unknown origin liver biopsy provides the only means of making this diagnosis.
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PMID:A prospective study of hepatic tuberculosis in 41 black patients. 365 66

Identical twin Caucasian boys, age 3 months, were seen with fever of unknown origin, hepatosplenomegaly, and pancytopenia. The diagnosis of familial erythrophagocytic lymphohistiocytosis (FEL) was suspected after examination of Twin A's bone marrow and confirmed by an open liver biopsy of Twin B. Twin A died shortly after diagnosis despite treatment with vincristine and prednisone. At autopsy, the diagnosis was confirmed. Twin B responded initially to a three-week course of weekly vincristine and daily prednisone, but symptoms soon recurred. In an effort to enhance delivery of chemotherapy to the active macrophage target, platelets were loaded with vinblastine and then administered intravenously to th patient every 7-10 days. There was an encouraging response reflected by the disappearance of symptoms and the return of peripheral blood count to the normal range, although increased number of histiocytes was still demonstrable in his bone marrow. After nine weeks, he lapsed completely and became refractory to treatment. He died of pseudomonas sepsis four months after diagnosis. This is the first known attempt to deliver a chemotherapeutic agent directly to the macrophages in treating this disease and represents an interesting concept that merits further exploration.
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PMID:Familial erythrophagocytic lymphohistiocytosis: treatment with vinblastine-loaded platelets. 719 95

Out of 23 cases with a final diagnosis of idiopathic fever, 20 had self-limited fever with complete resolution. Comparing the cases of self-limited fever with other groups of patients with fever of unknown origin, the following differences were apparent: compared with tumoral and collagen diseases, self-limited fever occurred more frequently below age 40, the difference being significant (p less than 0.01). Chills occurred more frequently in self-limited fever than in tumoral and collagen diseases, while the incidence was similar in infectious diseases. Infectious, tumoral and collagen diseases presented with significantly greater weight loss (p less than 0.01) than self-limited fever. A greater incidence of hepatosplenomegaly was noted in self-limited fever than in infectious diseases. Hemoglobin and erythrocyte sedimentation rate (ESR) were significantly higher in self-limited fever than in the other illnesses. The NBT test was positive, with a reduction superior to 30% in the six cases in whom it was performed. In nine cases various invasive procedures were utilized: radiology, biopsy, laparoscopy (two cases), and laparotomy (one case). The data on the present series of self-limited idiopathic fever support an infectious origin of the disease because of the following: absence of an age difference with the group with a demonstrated infectious cause; fever of less than two months duration in most cases; presence of chills and less incidence of weight loss, anemia, and elevated ESR in relation to the other groups of fever of unknown origin; a positive NBT test; and spontaneous evolution to complete resolution of the disease.
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PMID:[Self-limited idiopathic fever. A study of twenty cases (author's transl)]. 721 24

This report concerns the analysis of 100 cases of fever of unknown origin, defined according to the criteria of Petersdorf and Beeson. An etiological diagnosis could be reached in 77 cases, distributed as follows: infections, 32 cases; tumors, 14 cases; collagen diseases, 13 cases; various etiologies, 18 cases; and idiopathic, 23 cases of whom 20 had selflimited fever. There were 51 male and 49 female. The incidence of infectious diseases was significantly higher below age 40 when compared with the group of collagen diseases. Recurrent fever was common (43 cases) while continuous fever was unusual (3 cases). Recurrent fever was most frequent in neoplastic diseases, the difference in relation to the other groups being statistically significant (p less than 0.05). Chills, diffuse perspiration and myalgia were more frequent in infectious diseases (p less than 0.01) than in the other groups. Collagen diseases had significantly higher (p less than 0.05) white blood cell counts than neoplastic diseases, while the latter had significantly higher (p less than 0.01) serum lactic-dehydrogenase and alpha 2 globulin levels than infectious and collagen diseases. Hepatosplenomegaly was present more frequently in neoplastic than in infectious diseases. When evaluating diagnostic procedures it was noted that invasive techniques (arteriography, biopsy, laparoscopy, laparotomy) were essential to arrive to the final diagnosis in 47 cases, while noninvasive procedures (serological and immunological tests, bacteriology, conventional radiology, clinical course, and response to therapy) were sufficient in 22 cases. In eight cases the etiology of the fever could only be determined by post-mortem examination.
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PMID:[Fever of unknown origin. A study of 100 cases (author's transl)]. 721 37

A 17-year-old male had fever of unknown origin (FUO) for 3 months with positive c-reactive protein, lymph node swelling, and hepatosplenomegaly. Biopsy specimens of the liver and lymph node disclosed nonspecific inflammation. Lymph node swelling and hepatosplenomegaly subsided gradually, while vascular murmur and pulselessness appeared. Computerized tomography and magnetic resonance imaging showed thickened arterial wall, while angiography disclosed arterial narrowing. From these findings he was diagnosed to have Takayasu's arteritis. The therapy with prednisolone was effective. Takayasu's arteritis is rarely manifested by hepatosplenomegaly and lymphadenopathy in its prepulseless stage.
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PMID:Takayasu's arteritis in prepulseless stage manifesting lymph node swelling and hepatosplenomegaly. 764 21

Visceral leishmaniasis is infrequently reported in renal transplant recipients. A 40-year-old renal transplant recipient developed hepatosplenomegaly and pyrexia of unknown origin 5 months after transplantation. Visceral leishmaniasis was confirmed on bone marrow examination. The usual dose of antiparasitic therapy with stibogluconate sodium failed to eradicate Leishmania donovani. High-dose conventional therapy with stibogluconate sodium for an extended period of time was successful in the treatment of a relapse of leishmaniasis.
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PMID:Visceral leishmaniasis in a renal transplant recipient: diagnostic and therapeutic problems. 873 93


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