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)

Disseminated histoplasmosis has been recognized as a serious opportunistic infection in patients with acquired immunodeficiency syndrome (AIDS). However, cases reported in the literature have been predominantly in adult patients. Here we report an infant with AIDS who presented with fever, cough, rhinorrhea, hepatosplenomegaly, pancytopenia and coagulopathy, and died of respiratory failure. Autopsy revealed disseminated histoplasmosis involving multiple organs including lungs, intestines, liver, spleen, bone marrow, lymph nodes, kidneys, and meninges. The diagnosis was established based on histomorphology and confirmed by blood culture.
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PMID:Disseminated histoplasmosis in an infant with acquired immunodeficiency syndrome. 989 38

Imiglucerase, the recombinantly produced enzyme, is gradually replacing the human placental derived alglucerase in the treatment of gaucher patients. We describe the first case, to the best of our knowledge, of an anaphylactoid reaction to imiglucerase in a patient who tolerated alglucerase. The patient was diagnosed at the age of 2 4/12 years with anemia and hepatosplenomegaly. Over the years he had suffered from marked splenomegaly, thrombocytopenia and recurrent bleeding episodes. At the age of 24 he started treatment with imiglucerase. After 3 months of treatment, immediately after starting an infusion, he experienced flushing, cough, tachycardia, palpitation, chest pain and excessive sweating, which reoccurred on a consecutive administration. Substitution with alglucerase was tolerated well, with only mild rash when he was premedicated with benadryl. Immediate skin tests to alglucerase, imiglucerase and gelatin were negative. IgG against alglucerase was undetectable. The in vitro mast cell degranulation test was positive for alglucerase, imiglucerase heamaccel (a gelatin based plasma substitute, which is a component of imiglucerase). This sensitivity to imiglucerase but not to alglucerase, raises the question of future treatment for this patient, since the production of alglucerase may cease, once imiglucerase production will cover the need for replacement enzyme.
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PMID:Anaphylactoid reaction to imiglucerase, but not to alglucerase, in a type I Gaucher patient. 1038 90

A 65-year-old man who had an 8-year history of chronic lymphocytic leukemia was admitted to our hospital on February 19, 1998 because of high fever, dry cough, and weight loss. Laboratory data on admission included serum lactate dehydrogenase at 980 IU/l, CRP at 21.8 mg/dl, and soluble interleukin-2 receptor at 7,280 U/ml. The results of serological tests for Epstein-Barr virus (EBV) antibodies were as follows: EBV capsid antigen IgG 1:2560, EBV early antigen IgG 1:640, and EBV nuclear antigens 1:20. Computed tomography revealed diffuse interstitial pneumonia in both lungs, hepatosplenomegaly with multiple nodules, and enlarged intra-abdominal lymph nodes. In addition, Gallium-67 scintigraphy demonstrated abnormal accumulations. Although the patient initially responded well to combination chemotherapy, he eventually deteriorated and died on November 2, 1988, despite salvage chemotherapy. Postmortem needle biopsy specimens from the liver and spleen revealed diffuse proliferation of polymorphic large lymphoma cells. The lymphoma cells were positive for L-26, latent membrane protein 1, and EBV nuclear antigen, but negative for UCHL-1 and CD3, 5, 10, and 30. In situ hybridization procedures disclosed the presence of EBV-encoded small RNA in lymphoma cells. These findings suggested the possibility of association with EBV infection in some cases of Richter's syndrome.
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PMID:[Epstein-Barr virus associated Richter's syndrome accompanied by interstitial pneumonia]. 1039 Aug 89

A 54-year-old woman was admitted to Juntendo Izunagaoka Hospital on Aug. 29, 1998, after experiencing cough and fever for 19 days. Chest X-ray films disclosed infiltrates in the left lung field. The abnormal lung shadows progressed despite antibiotic therapy, and enlargement of superficial lymph nodes and hepatosplenomegaly developed. Peripheral blood examination disclosed cleaved lymphoid cells without granular cytoplasm. Anti-HTLV-I antibody titer was x320, and the monoclonal integration of HTLV-I provirus was confirmed by Southern blot analysis. Surface marker analysis of lymph node cells was positive for CD2, CD3, CD4, CD5, CD56, and HLA-DR. The above results yielded a diagnosis of adult T-cell leukemia. LSG-4 therapy alleviated the lung infiltrations and dyspnea. This case was considered unusual because of the expression of the natural killer cell marker CD56 on leukemic cells and the presentation of abnormal lung shadows possibly due to leukemic cell infiltration.
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PMID:[CD56-positive adult T-cell leukemia manifested by abnormal lung shadows]. 1069 96

A 29 day old male infant presented with a history of fever, cough, increasing respiratory distress and abdominal distension from the 10th day of life. Examination revealed failure to thrive, marked tachypnea, pallor, hepatosplenomegaly and harsh vesicular breath sounds. Chest skiagram showed extensive broncho-pneumonic changes. As the infant did not respond to antibiotics, he was investigated for tuberculosis. The gastric aspirate smear showed plenty of acid fast bacilli (AFB) and culture showed M. tuberculosis growth. Endometrial biopsy of the mother showed tuberculous granuloma and acid fast bacilli and culture of the aspirate from endometrium grew M. tuberculosis. The need for endometrial biopsy of mothers of infants with congenital tuberculosis is highlighted.
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PMID:Congenital tuberculosis. 1079 50

A 36 year old male was admitted in December 1997 to hospital with afternoon fever, malaise and hepatosplenomegaly. He also had a dry cough, dyspnoea and anaemia. Pneumonia caused by Pneumocystis carinii and human immunodeficiency virus (HIV) infection were documented. The HIV infection was confirmed in 1997 with 290,000 virus copies. The patient had been in the Mexican State of Chiapas which is known to be endemic for visceral leishmaniosis (VL) and localized cutaneous leishmaniosis (LCL). The visceral symptoms were diagnosed as VL and the causal agent was identified as Leishmania (L. ) mexicana. Identification of Leishmania was carried out by the analysis of amplified DNA with specific primers belonging to the Leishmania subgenus and by dot blot positive hybridisation of these polymerase chain reaction derived products with kDNA from the L. (L. ) mexicana MC strain used as probe. This is the first case in Mexico of VL caused by a species of Leishmania that typically produces a cutaneous disease form.
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PMID:Visceral leishmaniosis caused by Leishmania (L.) mexicana in a Mexican patient with human immunodeficiency virus infection. 1099 26

Childhood bronchial mucoepidermoid tumours (BMET) are rare. A 12-year-old boy with hepatosplenomegaly underwent liver biopsy which diagnosed amyloidosis. Chest radiograph and CT, performed for recurrent respiratory symptoms, identified a left lower lobe tumour, which was subsequently excised. Histology showed a BMET. A literature review reveals 51 reported cases of BMET in children. Common presenting symptoms include fever, cough and recurrent pneumonia. Diagnosis is often delayed and patients with recurrent respiratory symptoms should undergo CT or bronchoscopy. The association between amyloidosis and BMET in this case is unique and has not been previously described, but may be coincidental.
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PMID:Bronchial mucoepidermoid tumour in a child presenting with organomegaly due to secondary amyloidosis: case report and review of the literature. 1137 23

This is a case report of a 29 year old male with pneumocystis pneumonia and tuberculosis, and who was initially suspected of having HIV infection, based on risk factor analyses, but was subsequently shown to be HIV negative. The patient arrived at the hospital with fever, cough, weight loss, loss of appetite, pallor, and arthralgia. In addition, he was jaundiced and had cervical lymphadenopathy and mild heptosplenomegaly. He had interstitial infiltrates of the lung, sputum smears positive for Mycobacterium tuberculosis and Pneumocystis carinii, and stool tests were positive for Strongyloides stercoralis and Schistosoma mansoni. He was diagnosed as having AIDS, and was treated for tuberculosis, pneumocystosis, and strongyloidiasis with a good response. The patient did not receive anti-retroviral therapy, pending outcome of the HIV tests. A month later, he was re-examined and found to have worsening hepatosplenomegaly, pancytopenia, fever, and continued weight loss. At this time, it was determined that his HIV ELISA antibody tests were negative. A bone marrow aspirate was done and revealed amastigotes of leishmania, and a bone marrow culture was positive for Leishmania species. He was treated with pentavalent antimony, 20 mg daily for 20 days, with complete remission of symptoms and weight gain. This case demonstrates that immunosuppression from leishmaniasis and tuberculosis may lead to pneumocystosis, and be misdiagnosed as HIV infection. The occurrence of opportunistic infections in severely ill patients without HIV must always be considered and alternate causes of immunosuppression sought.
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PMID:Pneumocystis carinii pneumonia, pulmonary tuberculosis and visceral leishmaniasis in an adult HIV negative patient. 1150 79

Mediastinal masses in children are a heterogeneous group of asymptomatic or potentially life-threatening congenital, infectious, or neoplastic lesions that present complex diagnostic and therapeutic dilemmas. Some patients are asymptomatic; in others, the mass may compress mediastinal structures and cause sudden asphyxia. In these cases, close cooperation is needed among pediatric surgeons, anesthesiologists, intensivists, oncologists, and radiologists. The files of 45 children with mediastinal masses admitted between 1986 and 1999 to the Pediatric Intensive Care Unit (PICU) of Schneider Children's Medical Center of Israel were reviewed. Twenty-one were admitted for perioperative care, and 21 for emergency care, including 19 with respiratory distress. Five of the emergency care group had asphyxia and 10 needed assisted ventilation. Two children were admitted for evaluation and 1 for leukopheresis. The children admitted on an emergency basis had more clinical findings than the postoperative group: almost 80% had dyspnea and more than 45% had oxygen desaturation; 33% had cough and noisy breathing, and 25%, superior vena cava syndrome or hepatosplenomegaly. Eight patients (17.8%) had benign disease and 37 (82.2%) malignant disease. The patients with a benign mass were significantly younger than the patients with a malignant mass (p<0.005); in 5 cases (12.5%), a congenital anomaly presented as a mediastinal mass. Most of the malignant masses were of hematologic origin (40.5%), followed by neurogenic tumors (27%). Twenty-seven patients underwent surgery, including 6 emergency procedures (3 partial resections, 2 biopsies, 1 lymph node biopsy). There were no intraoperative or postoperative deaths. The present series emphasizes the complex care children with a mediastinal mass require. They should be treated in a tertiary center with a multidisciplinary approach.
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PMID:Mediastinal tumors in children: a single institution experience. 1204 17

We report a 52 years old male admitted for fever lasting one month, dry cough, headache and malaise. Initial laboratory work up showed an AST of 172 U/l, and ALT of 252 U/l, a GGT of 353 U/l and alkaline phosphatases of 952 U/l. An abdominal CAT scan disclosed a mild hepatosplenomegaly. A liver biopsy showed a granulomatous hepatitis. During the evolution, the patient had a left testicle swelling with darkening of the surrounding skin. A testicular ultrasound showed a bilateral orchiepidydimitis. The patient was treated with non steroidal anti-inflammatory drugs and fever subsided. Three months later, these drugs were discontinued and the patient remained asymptomatic and with normal laboratory values until 36 months of follow up.
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PMID:[Idiopathic granulomatous hepatitis with bilateral orchiepididymitis and skin eruption]. 1258 10


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