Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of B cell lymphoma with clinical and histological features of malignant histiocytosis was described. A 57-year-old male was admitted to Shinshu University Hospital because of transverse myelopathy. Five months before admission, he noticed urinary disturbance, which progressed to urinary obstruction. The following month, bilateral muscular weakness appeared in his legs. A few days later he could not stand up, and was admitted to a local hospital. Neurological examination revealed sensory disturbances below the level of Th12 in all modalities, and marked weakness and hyperreflexia in the lower limbs. A spinal tumor was suspected. However, myelography showed no abnormality. The patient's condition worsened and he became bed-ridden in February 1990. He was transferred to Shinshu University Hospital for further evaluation. On admission he was poorly nourished with fever, anemia, hepatomegaly, and bilateral pretibial pitting edema. No lymphadenopathy was observed. Neurological examination showed total sensory loss below the level of Th12, spastic paraplegia, hyperreflexia in the legs, and urinary obstruction. Laboratory findings revealed an elevated erythrocyte sedimentation rate, increased CRP, pancytopenia, and hypoalbuminemia. Serum level of IgG, IgA, IgM, LDH, ALP, GPT and total bilirubin were increased. CSF and MRI imaging of the spinal cord were normal. Proliferation of atypical histiocytes with marked erythrophagocytosis, which is a characteristic pathological feature of malignant histiocytosis, was observed in peripheral blood and aspirated bone marrow. Immunoenzyme staining of bone marrow using monoclonal antibody L-26, which is a B-cell marker, revealed B-cell lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of B-cell lymphoma with clinical and histological features of malignant histiocytosis]. 130 24

Two cases of atypical Kawasaki disease are reported. Case 1 was a five-month-old male infant admitted to this hospital with a 10-day's history of high fever. On examination, he appeared ill-looking and only hepatomegaly was noted. Laboratory studies showed leukocytosis, thrombocytosis, elevated ESR and pleocytosis in CSF. He was treated as sepsis with meningitis. Sudden death occurred on the eighth day of admission, and left coronary artery aneurysm with thrombosis was noted at autopsy. Case 2 was a four-month-old male infant referred to our hospital with fever and cervical lymphadenopathy of 11 day's duration, and unresponsive to antibiotics. Skin rash had developed after oxacillin injection. Echocardiogram, performed on the third day of admission, disclosed a 5-8 mm aneurysm of the left coronary artery and a 4 mm aneurysm of the right coronary artery. Before a specific diagnostic test for Kawasaki disease becomes available, we suggest that a possible diagnosis of Kawasaki disease and echocardiographic evaluation should be considered in case of (1) presence of partial criteria of Kawasaki disease with thrombocytosis; and/or (2) young infants with prolonged unexplained fever.
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PMID:Atypical Kawasaki disease: report of two cases. 151 14

Infantile Refsum's disease was diagnosed in three male patients, presenting with facial dysmorphia, retinitis pigmentosa, neurosensory hearing loss, hepatomegaly, osteopenia and delayed growth and psychomotor development. An elevated plasma phytanic acid concentration and a deficient phytanic acid oxidase activity in fibroblasts were found with an accumulation of very long chain fatty acids in plasma and fibroblasts. There were elevated pipecolic acid levels in plasma, urine and CSF, and abnormal bile acid metabolites in plasma. Deficient activity of acylCoA: dihydroxyacetone phosphate acyl transferase was found in thrombocytes and fibroblasts of these patients as well as an impaired de novo plasmalogen biosynthesis in fibroblasts. These biochemical abnormalities, previously described in the Zellweger syndrome, suggest multiple peroxisomal dysfunction in our patients.
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PMID:Infantile Refsum's disease: biochemical findings suggesting multiple peroxisomal dysfunction. 242 95

300 patients with sleeping sickness have been admitted, at the AHT clinic of Daloa, over a time period of 22 months. The sex ratio of the patients is 1.5 males for 1 female; the mean age is 25.5 years. The most frequent signs and symptoms observed by clinical examination are: fever (30%), nodes (86.3%), prurigo (43.3%), splenomegaly (15.3%), hepatomegaly (1%), headache (72.6%), vigilance and sleeping disturbances (68.7%), perioral reflexes (67.6%), cheiro-oral reflexes (64.3%), movement disorders consisting of tremor, choreo-athetosis movements, buccal dyskinesia or seizures (35%), motor palsy and gait disorders (15%), tonus disturbances (12.3%), sensitivity abnormalities (17%), endocrine disorders (16.3%), psychiatric symptoms (6.3%). According to CSF status, 261 patients have been classified in second period (P2). This group, although biologically well defined, is in fact a miscellaneous group of clinical signs and symptoms ranging from apparently normal patients to sleeping comatose and cachectic patients. 93% of the patients in this group have peripheral signs associated with neurological symptoms. They are as frequent in the first period as in the second period, with a statistical significance. This is an argument to think that the CNS is early affected in the course of the disease. The classification of the patients in groups of increasing neurological impairments, is in accordance with this hypothesis. 89% of the patients in the second period have only slight neurological signs. This explain how difficult it is for a physician to use melarsoprol in the treatment of all patients classified in second period.
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PMID:[The different present-day clinical picture of human African trypanosomiasis caused by T. b. gambiense. Analysis of 300 cases from a focus in Daloa, Ivory Coast]. 284 37

From December 1982 to June 1985, we diagnosed LAV/HTLV-III infection in 16 children of African origin living in Belgium or referred to one of the hospitals participating in this study. Diagnosis was proven in seven of them by isolation of virus of the LAV/HTLV-III group. In the nine others, LAV/HTLV-III infection was highly probable because of the presence of antibodies against the virus (seven subjects) or clinical and immunological evidence of immune deficiency associated with a parental history of LAV/HTLV-III infection (two subjects). Five of these children had a severe illness starting in the first months of life (range 20 days--4 months) and died within 4 months (range 19 days--10 months). Eight children presented later in life (mean age 35 months, range 2-66 months) with a milder and more chronic disease characterized by the presence of generalized lymphadenopathy (6/8), hepatomegaly (5/8), splenomegaly (5/8), interstitial pulmonary infiltration (4/8), parotid swelling (3/8), CSF lymphocytosis (3/5), diarrhoea without pathogen isolated (1/8) and fever (1/8). At least one of the parents of each child was of African origin. At the time of birth of their child two mothers and one father had an AIDS-related complex. After a mean period of 34 months (range 3-87 months) five fathers and six mothers had a symptomatic LAV/HTLV-III infection (AIDS or AIDS-related complex).
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PMID:LAV/HTLV-III infection in children of African origin: experience in Belgium. 294 77

Six infants with disseminated HSV had no mucocutaneous lesions at any time during the course of the illness. These infants presented with lethargy, poor feeding, apnea, acidosis, and hepatomegaly. The diagnosis of HSV was made by culturing the infant's oropharynx and blood, and the maternal cervix. Eight infants with HSV encephalitis had no skin, eye, or mucous membrane lesions. These infants presented with lethargy and low-grade fever, followed within 24 hours by the onset of focal partial motor seizures. The seizures were refractory to anticonvulsant therapy. The mean CSF white cell count was 131 cells/mm3;the glucose and protein concentrations were in the normal range. Brain biopsy was required for the early diagnosis of HSV encephalitis. These 14 cases presented 70% (14/20) of all infants with neonatal HSV diagnosed during the study period. HSV infection should be considered in infants with no mucocutaneous lesions who have signs usually associated with bacterial sepsis or who develop focal seizures during the first three weeks of life.
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PMID:Neonatal herpes simplex infection in the absence of mucocutaneous lesions. 706 32

Eighty six of 430 acute myeloblastic leukemia (AML) patients (20.0%) and forty of 173 acute lymphoblastic leukemia (ALL) patients (23.1%) had CD7 on their leukemia cells. CD7(+) AML occurred at a younger age than CD7(-) AML, and is more frequent in males. Hepatomegaly and central nervous system involvement were also more frequent in CD7(+) AML than in CD7(-) AML. The age of onset of CD7(+) ALL is also younger than that of CD7(-) ALL. Phenotypically, CD(+) AML expressed CD34, HLA-DR, and TdT more frequently than CD7(-) AML while CD7(+) ALL expressed CD13/33 more often than CD7(-) ALL cells responded most significantly to interleukin 3 (IL-3), whereas most CD7(-) AML cells responded more significantly to granulocyte macrophage-colony stimulating factor (GM-CSF) and/or granulocyte (G)-CSF than to IL-3. CD7(+)sCD3(-)CD4(-)CD8(-) ALL expressed G-CSF receptor and c-kit mRNA more frequently, which is not usual in other types of ALL. P-glycoprotein (P-gp)/multi-drug resistance gene (MDR1), thought to be expressed in hematopoietic stem cells, is expressed in CD7(+) AML and CD7(+)sCD3(-) CD4(-)CD8(-) ALL significantly more often than in CD7(-) acute leukemias and the CR rate and overall survival of CD7(+)AML was worse than CD7(-) AML. These data, collectively, suggest the close association of CD7(+) AML and CD7(+)sCD3(-)CD4(-)CD8(-) ALL, not only the common expression of CD7 itself but also because their phenotypical immaturity, cytokine receptor expression, P-gp/MDR1 expression and clinical manifestations including the frequent occurrence in males and the poor prognosis. We propose that CD7(+) acute leukemia is an hematopoietic stem cell leukemia which may be separate entity.
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PMID:Biological characteristics of CD7(+) acute leukemia. 872 5

Two children with Kawasaki disease (KD), a girl 3 yrs. and a boy 2.5 yrs., were described. The children met all criteria for the diagnosis of KD (fever, conjunctivitis, oral changes, extremity changes, rash, lymphadenopathy), and other diseases were excluded. In both children or one of them other clinical and laboratory findings occasionally detected in KD were observed: arthralgia, hepatomegaly and splenomegaly, slight elevation of transaminases and bilirubin level, slight elevation of CSF pleocytosis, sterile pyuria and hematuria. Electrocardiograms revealed sinus tachycardia and transient disturbances of heart repolarization. In both children changes in blood morphology and biochemical disturbances typical for inflammatory processes were noted. Thrombocytosis was seen in the 2nd week of the illness in the child in whom platelet counts were controlled. The child in whom diagnosis of KD was established in the first week of symptoms was treated with acetylsalicylic acid along with an oral penicillin. A child with retrospectively diagnosed KD was treated with antibiotics and corticosteroids. Clinical and laboratory findings of KD resolved in both children within 4-6 weeks without complications from coronary blood vessels.
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PMID:[Mucocutaneous lymph node syndrome (Kawasaki disease) in 2 children]. 892 38

Thirty-seven serum samples and five serum-CSF pairs collected from 42 acutely ill patients admitted to hospitals in Maharashtra (Bombay, Pune and Nasik); Orissa (Raurkela) and South Goa were referred to the National Institute of Virology (NIV), Pune (Maharashtra, India) for serodiagnosis. These patients had clinical manifestations of fever, hemorrhagic manifestations, hepatomegaly, shock syndrome and encephalopathy. Sixty-six percent of patients were children below ten years of age. Serological investigations revealed infection to dengue virus in all the patients as indicated in detection of IgM antibodies predominantly to dengue viral antigens. An important outcome of the study is that 10 patients referred to NIV with a provisional diagnosis of viral encephalitis proved to be dengue.
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PMID:Hemorrhagic manifestations and encephalopathy in cases of dengue in India. 918 55

The Severe Chronic Neutropenia International Registry (SCNIR) was established in 1994 following four phase I/II and one phase III clinical trial on the use of filgrastim (recombinant human granulocyte colony-stimulating factor [r-metHuG-CSF]) as a treatment for severe chronic neutropenia (SCN). A primary purpose of the SCNIR is to monitor SCN patients treated with filgrastim for adverse events that might occur over time. As of December 31, 2000, 832 patients with SCN (384 congenital, 160 cyclic, 288 idiopathic) were enrolled. Clinical trial and Registry data show that filgrastim is an effective treatment for SCN; more than 90% of patients treated respond with normalization of blood neutrophil counts. The SCNIR has collected data on bone pain, splenomegaly, hepatomegaly, thrombocytopenia, osteopenia/osteoporosis, vasculitis, glomerulonephritis, growth and development, pregnancy and fertility, leukemic transformation, and mortality. Analysis of data from patients who received filgrastim for up to 11 years did not identify any adverse events associated with increased duration of treatment.
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PMID:Risk and benefit of treatment of severe chronic neutropenia with granulocyte colony-stimulating factor. 1195 97


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