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)

Histoplasmosis is a fungal disease caused by the dimorphic fungus Histoplasma capsulatum, recognized as an AIDS-defining illness since the Center for Disease Control's revision criteria in 1985. This infection is reported to be present in 5-20% of AIDS patients, and in 95% of the cases it is manifested in its disseminated form. Serum antibodies and/or antigen research can make diagnosis, but the demonstration of the agent by culture or histopathological examination remains the gold standard methods. Co-infections in patients with AIDS are well known; however, reports on disseminated tuberculosis and histoplasmosis are scarce. The authors report the case of a female patient who presented a short-course history of weight loss, fever, and mild respiratory symptoms, with hepatosplenomegaly and lymphadenopathy. Laboratory workup called attention to anemia, altered liver, canalicular enzymes, liver function tests, high titer of lactate dehydrogenase (LDH), and pulmonary nodules on thoracic computed tomography. Incidental finding of yeast forms within the leukocytes during a routine blood cell count highlighted the diagnosis of histoplasmosis. The patient started receiving amphotericin B but succumbed soon after. The authors emphasize the possibility of this co-infection, the diagnosis of severe infection through the finding of yeast forms within peripheral leukocytes, and for the high titer of LDH in aiding the differential diagnosis.
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PMID:Co-infection of disseminated histoplasmosis and tuberculosis in an AIDS patient. 3152 18

Paroxysmal cold hemoglobinuria is a rare form of autoimmune hemolytic anemia caused by the Donath-Landsteiner autoantibody. The condition is characterized by the presence of an IgG biphasic hemolysin with specificity to the P blood group antigen. The antibody biphasic action may be demonstrated in the Donath-Landsteiner test. While paroxysmal cold hemoglobinuria can be manifested at any age, it typically appears in children following a viral upper respiratory syndrome or immunization, though rarely. This report describes a 23-months old girl presented with 5 days history of fever, erythrocytopenia, leukocytosis and occurrence of dark urine. On admission, the physical examination showed pallor, no scleral icterus, a mild hyperemic throat and no hepatosplenomegaly. The investigations revealed severe anemia with hemoglobin of 44g/L, increased reticulocyte count (10.67%), elevated lactate dehydrogenase (2603IU/L), decreased serum haptoglobin (0.159g/L), normal G6PD. Direct antiglobulin test was positive with C3d and C3c complement components only. Direct and indirect Donath-Landsteiner tests were positive. The girl was treated with a intravenous immunoglobulin infusion and Cefotaxime. She received transfusion of red blood cells, crossmatched, although P antigen untyped. Despite this in vitro serological incompatibility she had a hemoglobin increase. The patient was discharged in stable condition on the seventh day following admission. Paroxysmal cold hemoglobinuria is a hemolytic anemia for which a specific diagnostic test is available. Timely recognition of the disease by pediatricians is crucial as well as the highly skilled hospital blood bank staff performing Donath-Landsteiner testing.
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PMID:A rare case report of autoimmune haemolytic anemia in a female child due to a Donath-Landsteiner antibody. 3224 65

Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia with thrombocytopenia. In addition to the primary TMA syndromes, microangiopathic hemolytic anemia with thrombocytopenia can be seen in many systemic diseases. Transplant associated TMA (TA-TMA) affects patients following stem cell or solid organ transplant. A 48-year-old male who underwent autologous stem cell transplant for nonsecretory multiple myeloma was admitted to our hospital with worsening anemia, thrombocytopenia, renal dysfunction and hepatosplenomegaly. Initial blood work revealed rare schistocytes and normal lactate dehydrogenase and haptoglobin levels. He underwent an extensive workup looking for an infectious, inflammatory or malignant etiology but a definitive diagnosis could not be reached. Over his prolonged stay at the hospital, he suffered from multiorgan failure and eventually passed away. An autopsy revealed TMA involving all clinically affected organ systems and was deemed to be the cause of his demise. The absence of typical blood work suggestive of hemolysis does not rule out a diagnosis of TA-TMA. Knowledge of this rare disease entity will help physicians identify and treat this life-threatening condition early and effectively.
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PMID:A Fatal Case of Thrombotic Microangiopathy Without Schistocytosis and Absent Biochemical Markers of Hemolysis. 3226 9


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