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)

From July, 1974 to February, 1978, we managed 12 infants with listeriosis. This infection presented in two distinct forms: an early-onset type (nine patients), often representing a congenital infection following maternal illness, and a late-onset type in which the patient presented with meningitis (three patients). Of our nine infants with early-onset disease, three died, three developed permanent sequelae, and only three were normal at follow-up. Appropriate early management in the perinatal period may improve the outlook in this condition. Affected infants were often premature and had pneumonia, rash, and hepatosplenomegaly at birth. Prenatal clues to the diagnosis included maternal fever, abdominal pains, and leukocytosis with meconium staining of the preterm amniotic fluid. Examination of gastric aspirate at birth showed gram-positive coccobacilli. Antibiotic therapy should be started prenatally and continue for three weeks after birth to prevent recurrences of the late-onset type. This late-onset disease presented as meningitis after the second week of life and responded well to antibiotics. Our three patients recovered without sequelae.
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PMID:Perinatal listeriosis--a review of twelve patients. 43 5

Histoplasma meningitis (HM) has been reported to occur primarily in association with disseminated histoplasmosis (DH). We report a case of histoplasma meningitis occurring in a patient with common variable hypogammaglobulinemia (CVH) in which no manifestations of DH were observed. L. L., a 66-year-old Caucasian male, clerical worker, developed occasional episodes of dizziness and tinnitus in mid-1971. During 1972, increasing frequency of these episodes and gradually progressive confusion were noted. In January 1973, vomiting, forther confusion, obnubilation, and a left central facial paresis developed and he was hospitalized. Physical examination revealed no pulmonary abnormalities, lymphadenopathy or hepatosplenomegaly. Over the ensuing 6-week evaluation, there was occasional fever to 38.5 degrees C. Chest roentgenogram was normal. Cerebral angiography suggested a mass in the left cerebellar hemisphere. EEG was diffusely slow. Multiple CSF examinations revealed: Glucose 7-18 mg/with a normal blood glucose, protein 109-256 mg/and cells 66-140 (95 + % mononuclear). Histoplasma capsulatum was cultured from CSF but not from sputum, urine, blood or bone marrow. Skin tests for PPD, histoplasmosis, coccidiodomycosis, blastomycosis, mumps, dinitrochlorobenzene and streptokinase-streptodornase were negative then and 6 months later. Histoplasma serum antibody was absent. Immunoglobulin analysis revealed IgG 430 mg %, IgA 46 mg %, and IgM 35 mg %, which with the history and skin test results suggested CVH. Treatment with 2.51 gm of amphotericin B given intravenously over a 3-month period resulted in complete reversal of all neurologic signs and clearing of the confusion. The remission has been maintained for two years. This case represents a primary infection of the CNS by histoplasma. The relationship between the HM and the CVH will be discussed.
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PMID:Histoplasma meningitis with common variable hypogammaglobulinemia. 61 43

Between October, 1985, and February, 1987, 28 (8.7%) cases of scrub and murine typhus were diagnosed among 320 children with greater than or equal to 1 week history of obscure fever. Scrub typhus is a rural disease and characterized by fever, tachypnea and hepatosplenomegaly. Skin rash was rare and eschar was absent. Four patients had pneumonia and two had meningitis. Murine typhus, more an urban disease, was milder and half the patients presented exclusively because of night fever. Slightly enlarged liver and skin rash were the only significant physical signs. Lacking the classical textbook presentations, both rickettsioses often were missed or diagnosed as enteric fever. Recognition is important because patients with either disease respond well to treatment with chloramphenicol or doxycycline.
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PMID:Scrub and murine typhus in children with obscure fever in the tropics. 204 66

The predominantly heterosexual transmission of human immunodeficiency virus (HIV) in Africa suggests that pediatric acquired immunodeficiency syndrome (AIDS) could develop into a significant child health problem in this region. To assist clinicians in recognizing HIV infection in African children, the clinical features of 185 children with symptomatic HIV-related disease diagnosed at the 2 central hospitals in Harare, Zimbabwe, from April 1986-July 1987 were enumerated. In this period, 185 such cases were diagnosed. 83 (47%) involved children 0-12 months of age and another 61 (35%) represented children 13-24 months old. The male/female ratio was 1.0:1.03. The most frequently recorded clinical feature (52% of cases) was generalized lymphadenopathy, with or without hepatosplenomegaly. 45% of HIV-infected children presented with respiratory symptoms and pulmonary infiltrates on chest x-ray. Failure to thrive was present in 38% of cases. Also relatively common were hepatomegaly and splenomegaly (35% and 26%, respectively). Chronic, recurrent diarrhea was present in 21%. Less frequently observed (under 10% of cases) clinical findings were maculopapular eczematoid rashes, parotid swelling, chronic suppurative otitis media, chronic mucopurulent rhinitis, meningitis, and encephalopathy. 3 main clinical modes of presentation were identified--children with failure to thrive or marasmus in association with chronic diarrhea and developmental delay, those with generalized lymphadenopathy and hepatosplenomegaly, and children who present with chronic cough with pulmonary infiltrates on chest x-ray.
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PMID:Clinical presentation of symptomatic human immuno-deficiency virus in children. 226 23

Streptobacillus moniliformis is an uncommon human pathogen contracted from exposure to rodents. It usually produces a mild, protracted illness (rat-bite fever, Haverhill fever, erythema arthriticum epidemicum) that has either a favorable response to antibiotic therapy or spontaneously resolves. This report describes a fatal case of Streptobacillus moniliformis in an infant bitten by a wild rat. The autopsy findings included an interstitial pneumonia, fibrinous endocarditis, mild mononuclear meningitis, hepatosplenomegaly and lymphadenopathy, erythrophagocytosis, and sinusoidal mononuclear cell infiltrates in regional lymph nodes and the liver. To the authors' knowledge, this is the first report of the autopsy pathology findings of this agent.
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PMID:Fatal Streptobacillus moniliformis infection in a two-month-old infant. 271 62

Neonatal onset multisystem inflammatory disease is a rare disorder first described by Lorber in 1973. An additional 29 cases have been recorded. Two patients are described here, one with a 17 year follow-up. The typical features are a rash, fever, adenopathy, hepatosplenomegaly, and a severe, deforming arthropathy predominantly affecting large joints. The most striking feature is the onset in the neonatal period. Other associated features include inflammation, chronic meningitis, anemia, and persistent leukocytosis. Most, if not all, patients develop bizarre epiphyseal radiographic findings that are virtually pathognomonic. This disease is distinct from Still disease.
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PMID:NOMID--a neonatal syndrome of multisystem inflammation. 278 38

Familial erythrophagocytic lymphohistiocytosis, a rare disorder affecting infants, is characterized by a visceral infiltration of histiocytes and lymphocytes resulting in rapid death. It has recently been reported that use of epipodophyllotoxin, VP 16-213, could induce a complete remission of the disease. Such treatment does not, however, prevent fatal CNS relapse. Four patients with the characteristic features of the disease--fever, hepatosplenomegaly, pancytopenia, low plasmatic fibrinogen level, hyperlipidemia, and histiocytic meningitis--are described. These patients were treated with a combination therapy including systemic administration of VP 16-213, steroids, and intrathecal methotrexate followed by cranial irradiation after the age of 12 months. The four patients achieved complete remission of the disease after clearing of the CNS localization. Two patients had secondary relapses, but all four patients have had a disease-free survival exceeding 12 months. All patients have been in remission of the disease for 27, 20, 16, and 13 months, respectively, after disease onset without major setbacks from the treatment. This combination therapy appears to be a promising approach toward long-term remission of the disease.
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PMID:Treatment of four patients with erythrophagocytic lymphohistiocytosis by a combination of epipodophyllotoxin, steroids, intrathecal methotrexate, and cranial irradiation. 383 78

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly, but flared up after several years. Skin lesions appeared in the form of erythema, papules, or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was 0%-2%. The serum lactate dehydrogenase (LDH) value was within normal range and was not associated with hypercalcemia; lymphadenopathy, hepatosplenomegaly, and bone marrow infiltration were very slight. In most cases, hypergammaglobulinemia was seen, and in one case, monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their anti-ATLA antibodies were positive; none had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness and died of cryptococcus meningitis. Based on clinical and pathologic differences, we believe that these cases should be distinguished from typical ATL cases for the purposes of prognosis and treatment.
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PMID:A proposal for smoldering adult T-cell leukemia: a clinicopathologic study of five cases. 622 22

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly but flared up after several years. Skin lesions appeared in the form of erythema, papules or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was from 0% to 2%. The serum lactic dehydrogenase value was within normal range, and was not associated with hypercalcemia, lymphadenopathy, or hepatosplenomegaly, and bone marrow infiltration was very slight. In most cases, hypergammaglobulinemia was seen, and in one case monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their sera were positive for anti-ATL-associated antigen antibodies. None of them had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness, and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness, and died or cryptococcus meningitis. These cases were clinically and pathologically different from typical ATL cases already reported, and we feel it necessary to make distinctions from the viewpoints of prognosis and treatment. In discussing these cases, we compared smoldering ATL with typical ATL, and deliberated upon the causes of both.
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PMID:A proposal for smoldering adult T-cell leukemia--diversity in clinical pictures of adult T-cell leukemia--. 660 27

We studied two children who died of suspected familial erythrophagic lymphohistiocytosis. The first child, a 7-year-old boy, had fever, diffuse pulmonary infiltrates, bilateral choroidal effusions, diabetes insipidus, and marked meningitis with increased cerebrospinal fluid protein. Ocular histologic studies disclosed diffuse choroidal infiltrates consisting of atypical mononuclear cells with foamy cytoplasm. Similar histologic changes were seen in the lungs, liver, brain, and kidneys. The second child, the 5-year-old male cousin of Patient 1, had fever, hepatosplenomegaly, pulmonary infiltrates, and cerebrospinal fluid pleocytosis. Histologic changes were similar to those in the first case, but viral particles of the herpes simplex virus group were noted in brain tissue as well. The findings of virus particles in this histiocytic proliferative disease suggested a defect in the inflammatory response in these patients.
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PMID:Two cases of suspected familial erythrophagocytic lymphohistiocytosis. 724 97


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