Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report the cases of five children in whom kala-azar was undiagnosed at first instance. In these cases, the diagnosis was misled because of incomplete features (lack of fever, splenomegaly or hypergammaglobulinemia) an associated disease (hydatic cyst of the liver) or a complication dominating the clinical pattern (septicemia, staphylococcus respiratory infection). In one case, the patient was explored in order to diagnose portal hypertension.
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PMID:[Misleading forms of visceral leishmaniasis in children. Apropos of 5 cases]. 194 39

A case of malignant histiocytosis in a two year old boy is reported. His main clinical features were fever, lymphadenopathy, hepatomegaly and splenomegaly. Lymph node biopsy showed a sinusoidal type of lymph node infiltration, histiocytes of malignant aspect and erythrophagocytosis. Liver infiltration with tumoral cells was demonstrated by needle biopsy. The clinical evolution was rapidly progressive and after six months of chemotherapy he died of intercurrent respiratory infection.
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PMID:[Malignant histiocytosis in children]. 248 97

Between 1976 and 1979 a myeloproliferative disease associated with cells monosomic for chromosome number 7 in the bone marrow was seen in six boys aged 5 1/2 months to 8 years (median 10 months). Presenting features included hepatosplenomegaly (5/6), respiratory infections (4/6), pallor (2/6) and skin infections (1/6). Haematological features included a leucoerythroblastic anaemia with leucocytosis and thrombocytopenia, and a hyperplastic marrow with a slight excess of blasts. Fetal haemoglobin was normal in four patients and mildly raised in the other two. Neutrophil function tests showed defective chemotaxis with reduced killing, despite a normal NBT test. Cytogenetic analysis of the marrow showed a preponderance of cells with monosomy 7; the blood lymphocytes were cytogenetically normal. In three patients the disease progressed to acute myeloid leukaemia (AML) after 3 weeks to 23 months; the only patient who remitted did so in response to 6-mercaptopurine and prednisolone, but relapsed 16 months later. A fourth child developed massive splenomegaly which initially responded to 6-mercaptopurine and prednisolone, but progressed to myelofibrosis 11 months later. A fifth child died from anaemia and respiratory infection without progression to leukaemia and the sixth patient has not yet developed leukaemia. Monosomy 7 is the diagnostic criterion of one of the more common myeloproliferative states in childhood and carries a high risk of progression to AML. The acute phase is usually resistant to chemotherapy, but even in responsive cases treatment does not result in elimination of the abnormal clone. Allogeneic bone marrow transplantation should be considered in cases with a suitable donor.
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PMID:Monosomy 7 in childhood: a myeloproliferative disorder. 694 67

The pattern of infection was compared in 139 children with sickle cell-hemoglobin C (SC) disease and in 250 control children with a normal hemoglobin (AA) genotype ascertained at birth and followed prospectively for periods of zero to six years. Both infection incidence rates and survival curve analysis indicated highly significant increases in serious infection among children with SC disease. Respiratory infection and gastroenteritis were the most common infections, but only respiratory infections were significantly more frequent in SC disease. Pneumococcal bacteremia was confined to the SC group. No hematologic differences were apparent between SC patients with and without a history of serious infection, but infection was significantly more common in patients manifesting early splenomegaly.
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PMID:Pneumococcal and other infections in children with sickle-cell hemoglobin C (SC) disease. 709 8

Murine gammaherpesvirus (MHV-68) causes an acute respiratory infection followed by a latent infection in B lymphocytes. In the first 2-3 weeks after infection mice develop a marked splenomegaly, where the spleen cell number increases by 2-3 fold. Cytofluorimetric analysis during splenomegaly revealed an increase in numbers of B lymphocytes and of both CD4+ and CD8+ T lymphocytes. The largest increase relative to uninfected spleens was in the CD8+ population. The number of latently infected cells in the spleen peaked at day 10 post-intraperitoneal infection, then declined to 1/10(6)-1/10(7) cells per spleen. Depletion of CD4+ T lymphocytes prevented the splenomegaly and greatly reduced the peak infective centre level, while having no effect on the long-term of latently infected cells. Given the similarity between MHV-68-induced splenomegaly and Epstein-Barr virus-induced infectious mononucleosis, these data highlight the usefulness of MHV-68 as a mouse model for the study of gammaherpesvirus immunology and pathobiology.
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PMID:Murine gammaherpesvirus-induced splenomegaly: a critical role for CD4 T cells. 862 50

Psittacosis, also referred to as ornithosis, is a disease primarily of birds, which may be transmitted to humans. Psittacosis is caused by Chlamydia psittaci, an obligate intracellular parasite found worldwide. Humans are infected with C. psittaci when the organism enters the blood stream, usually through inhalation of dried excrement from diseased birds or through wound contamination with infected avian secretions. C. psittaci replicates in the liver and spleen and infects the lung and other organs hematogenously.1 The clinical manifestations of human psittacosis range from a mild respiratory infection to a severe systemic illness.1,2 Symptoms are frequently described as flu-like with fever, headache, body aches, and dry or productive cough. Sore throat, chest pain, abdominal pain, vomiting, and diarrhea are variably present. Physical findings may include a pulse-temperature dissociation, localized lung crackles, hepatomegaly, splenomegaly, and a pale macular skin rash. Chest radiographs may demonstrate lesions that are atelectatic, patchy, miliary, nodular, or consolidated in one or both lungs. White cell counts, erythrocyte sedimentation rates, and liver function tests are usually normal. In severe illness, signs and symptoms of liver dysfunction, neurological impairment, and respiratory and renal failure may be present. Since 1879 when psittacosis was recognized as a disease entity, cases have been reported in North and South America, Europe, Asia, and Australia. However, reports of psittacosis in Africa have been rare. An Ethiopian group, studying community-acquired pneumonia, published what they claimed to be the first report of psittacosis in Africa in 1994.3 The report published here is believed to be the first documented case of human psittacosis in Egypt.
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PMID:Psittacosis in Egypt: A Case Study. 981 79

The murine gamma-herpesvirus MHV-68 causes an acute, transient pneumonitis, followed by an infectious mononucleosis (IM)-like illness with splenomegaly, widespread latent infection of B lymphocytes and an expansion of Vbeta4+ CD8+ T cells. CD8+ T cells specific for an H-2Db-restricted epitope were prominent during the acute respiratory infection, but their prevalence declined rapidly during the mononucleosis. In contrast, CD8+ T cells specific for an H-2Kb-restricted epitope, apparently expressed by virus-infected B lymphocytes, were most numerous during the mononucleosis illness and were maintained at relatively high frequencies thereafter. The prevalence of all peptide-specific CD8+ T cells decreased during the expansion of the Vbeta4+ CD8+ population, which did not recognize any peptide epitopes identified and was apparent also in an MHC class I-deficient environment. The CD8+ T cell population recognizing productively infected epithelial cells thus differed substantially from that responding during the IM illness.
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PMID:Changing patterns of dominance in the CD8+ T cell response during acute and persistent murine gamma-herpesvirus infection. 1022 71

Epstein-Barr virus (EBV) is a ubiquitous human gamma-herpesvirus that establishes life-long latency and is associated with lymphoproliferative disorders and the development of several malignancies. EBV infection is frequently, but not always, associated with the development of a syndrome termed infectious mononucleosis. The recent isolation and characterization of a murine gamma-herpesvirus, MHV-68 (gammaHV-68) has provided the first small animal model for studying immunity and pathogenesis of a gamma-herpesvirus in its natural host. MHV-68 has important biological and genetic similarities with the human gamma-herpesviruses. Following intranasal infection of mice with MHV-68, an acute respiratory infection in the lung develops and is cleared, followed by the establishment of latency. Similar to EBV, MHV-68 latency is largely established in B cells, although other cell types can be latently infected. The establishment of latency correlates with a prominent splenomegaly, polyclonal B cell activation with associated autoantibody production, and CD8+ T cell-dominated peripheral blood lymphocytosis, in many aspects mirroring EBV-induced infectious mononucleosis. There are key differences in the MHV-68- and EBV-induced CD8+ T cell responses however. Whereas the expanded CD8+ T cells associated with EBV-induced mononucleosis are largely the outgrowth of T cells responding to lytic viral epitopes elicited during the acute phase of the response, the CD8+ T cell lymphocytosis associated with MHV-68-induced infectious mononucleosis is dominated by an oligoclonal population of T cells expressing Vbeta4+ T cell receptors that are not reactive to acute viral epitopes. The focus of this article will be to highlight the similarities and differences in the infectious mononucleosis syndrome associated with human and murine gamma-herpesviruses.
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PMID:A mouse model for infectious mononucleosis. 1201 60

Francisella tularensis can cause severe disseminated disease after respiratory infection. The identification of factors involved in mortality or recovery following induction of tularemia in the mouse will improve our understanding of the natural history of this disease and facilitate future evaluation of vaccine candidate preparations. BALB/c mice were infected intranasally with the live vaccine strain (LVS) of F. tularensis subsp. holarctica and euthanized at different stages of disease to analyze the induction of immune molecules, gross anatomical features of organs, bacterial burdens, and progression of the histopathological changes in lung and spleen. Tissue-specific interleukin-6 (IL-6), macrophage inflammatory protein 2, and monocyte chemotactic protein 1 were immune markers of mortality, while anti-LVS immunoglobulin M and IL-1beta were associated with survival. Moribund mice had enlarged spleens and lungs, while surviving mice had even more prominent splenomegaly and normal-appearing lungs. Histopathology of the spleens of severely ill mice was characterized by disrupted lymphoid follicles and fragmented nuclei, while the spleens of survivors appeared healthy but with increased numbers of megakaryocytes and erythrocytes. Histopathology of the lungs of severely ill mice indicated severe pneumonia. Lungs of survivors at early time points showed increased inflammation, while at late times they appeared healthy with peribronchial lymphoid aggregates. Our results suggest that host immune factors are able to affect bacterial dissemination after respiratory tularemia, provide new insights regarding the pathological characteristics of pulmonary tularemia leading to systemic disease, and potentially identify immune markers associated with recovery from the disease.
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PMID:Identification of immunologic and pathologic parameters of death versus survival in respiratory tularemia. 1802 95

Immunocytoma is a non-Hodgkin's indolent evolution B cell lymphoma. It accounts for approximately 1-3% of non-Hodgkin's limphomas and usually onsets in adults aged over 50 years old. It manifests as lymphadenopathy, splenomegaly, hepatomegaly and lymphcytosis in 15 -30% of cases and is rarely seen with pulmonary involvement. Monocloncal peaks of serum immunoglobulin often occur. These are IgM and rarely IgA. We present as an example a male patient aged 52 years old, with recurrent respiratory infections. Clinical work -up identified an immunocytoma IgA stage IV. Diagnosing an indolent lymphoma, we prophylactic polyspecific human immunoglobulin to treat the respiratory infection. Evidence of lymphoma progression leads us to prescribe combined cyclo- phosphamide (C), vincristine (V), prednisone (P) e rituximab (R) (CVP-R), which has obtained a partial response over two years.
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PMID:[Immunocytoma IgA. Case report]. 1914 95


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