Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report, which describes an epidemic of St. Louis encephalitis in northwestern Mexico, was prompted by the apparent infrequency of previous Mexican outbreaks of this disease. The site of the epidemic was the Municipality of Hermosillo in the State of Sonora. This municipality, with a population of about 273,000, is situated in a hot flat area which is naturally dry but which is also well-irrigated. Here, over an eithy-week period, 51 persons became ill and were hospitalized with symptoms including fever, vomiting, stiff neck, lethargy, and increased cell/protein levels in their cerebrospinal fluid. Sera from 12 of these patients were tested for SLE antibodies by hemagglutination inhibition, complement fixation, and neutralization. The results of these tests were positive and showed a fairly high degree of correlation. In addition, paired serum samples showed a significant rise in SLE antibody titers. Over half of the cases occurred in the City of Hermosillo, the municipal seat; the remainder occurred in 13 outlying areas, most of them quite nearby. Virtually all of the city dwellers affected were residents of low-income districts. The rate of hospital cases was roughly 19 per 100,000 inhabitants, and the case-fatality rate among these cases was approximately 20 per cent. The ratio of male to female patients was 1.3 to 1; sixty-nine per cent of the patients were under 16 years of age.
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PMID:St. Louis encephalomyelitis in Hermosillo, Sonora, Mexico. 121 40

Systemic lupus erythematosus was diagnosed in a 3-year-old, male German Shepherd Dog. Clinical signs included lethargy, partial anorexia, fever, joint swelling, and skin and oral ulceration. The diagnosis was confirmed on the basis of an antinuclear antibody titer of 1:640 and immunofluorescence for immunoglobulin G at the dermal-epidermal junction in skin biopsy specimens. Treatment with prednisone and cyclophosphamide failed to induce remission. Intensive plasmapheresis-immunoadsorption, using purified Staphylococcus protein A in combination with low-dose prednisone therapy, resulted in sustained remission.
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PMID:Plasmapheresis-immunoadsorption for treatment of systemic lupus erythematosus in a dog. 633 55

M. pneumoniae is a common cause of pneumonia. The diagnosis is suspected when the patient presents with symptoms suggesting primary atypical pneumonia including cough, fever, chills, headache, and malaise in association with a segmental or subsegmental pulmonary infiltrate(s), the white blood cell count is normal or only slightly elevated, and the Gram stain of the sputum (if any can be obtained) reveals polymorphonuclear leukocytes and few bacteria. The diagnosis is more difficult when the patient presents with symptoms not suggestive of pneumonia including lethargy, dyspnea, and a 1- to 4-week history of shortness of breath without cough or fever in association with diffuse reticulonodular or interstitial pulmonary infiltrates. The disease in the previously healthy host is usually benign and self-limiting. However, the course is shortened by the administration of tetracycline derivatives or erythromycin. M. pneumoniae pneumonia can occur in association with other diseases including sickle cell anemia, sarcoidosis, systemic lupus erythematosus, Hodgkin's disease, and various other immunodeficiency states. In these patients mycoplasma pneumonia can be very serious. Although there is no pathognomonic clinical or radiographic presentation, careful consideration of epidemiologic, clinical, laboratory, and radiographic data are usually sufficient to suggest the diagnosis in most patients.
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PMID:Mycoplasma pneumonia. 676 79

The records of 37 patients with systemic lupus erythematosus (SLE) followed at The Children's Hospital of Philadelphia between 1968 and 1978 were reviewed for evidence of central nervous system (CNS) involvement. Criteria for CNS involvement included evidence of organic brain syndrome, electroencephalographic abnormalities with symptoms referable to CNS, or objective neurologic signs. Sixteen of 37 children had CNS involvement (43%). Thirteen patients had CNS involvement at the onset of SLE. Three patients had late onset CNS manifestations 1 to 2 years after the diagnosis of SLE. The most frequently observed symptoms were headache, behavior disorder, lethargy, diplopia, blurred vision, memory alteration, dizziness, and alteration of consciousness. The most frequently observed neurologic signs were seizures, cranial nerve palsy, ataxia, papilledema, nystagmus, meningitis, tremor, rigidity, cortical blindness, and coma. Neuropsychiatric manifestations included organic brain syndrome, functional psychosis, and personality disorder. Laboratory tests showed elevated cerebrospinal fluid opening pressure and protein, negative cultures, and abnormal electroencephalograms and computerized axial tomography scans. Fourteen of 16 children with CNS manifestations are alive. Thirteen had a mean IQ of 89 by the Wechsler Intelligence Tests. Twelve are in educational programs. One required long-term psychiatric care. A residual neurologic abnormality, a seizure disorder, was present in 3. CNS involvement with SLE in children carries a favorable prognosis.
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PMID:Central nervous system involvement in childhood systemic lupus erythematosus. 731 16

Most attempts at developing a model of autoimmune inner ear disease have focused on the immunization of healthy animals with cochlear tissue. We have chosen an alternate route of studying this entity utilizing the MRL-lpr/lpr (Lupus) mouse, an animal known to spontaneously develop multisystemic, organ nonspecific autoimmune disease. We report on the auditory pathology found in animals at early stages of this systemic disease. At the onset of clinical signs of illness (cachexia, weight loss, lethargy) animals were sacrificed and their cochleas and kidney prepared for morphologic analysis. Significant pathology was seen in the MRL/lpr animals involving the basal and middle turns of the cochlea which could not be correlated with the presence or degree of glomerulonephritis. Findings included outer and inner haircell degeneration, strial edema and degeneration, and an acellular infiltrate in the tunnel of Corti. Cochlear pathology was not found in control animals. Thus, at early stages of systemic disease, MRL/lpr mice manifest significant cochlear pathology not seen in control animals. The implications of these results with regard to the pathogenesis of these lesions as well as their clinical relevance are discussed.
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PMID:The MRL-lpr/lpr mouse: a potential model of autoimmune inner ear disease. 847 29

Bilateral paramedian thalamic infarction is rare. The suggested mechanism is occlusion of a central unpaired thalamic perforating artery--an anatomic variant. In the few existing reports of this condition, the diagnosis was based on computed tomography (CT) or magnetic resonance imaging (MRI) findings alone. Other causes of thalamic lesions were not ruled out, and there was no angiographic demonstration of the presumed variant artery. We present a case of a 48-year-old man with a bilateral thalamic infarction seen on CT and MRI. Initial neurological examination revealed lethargy, severe combined motor and sensory aphasia, and a mild upward gaze limitation. The patient had no focal motor deficits. After 24 hours, the patient was more alert and his speech became more fluent, but Korsakoff-type amnesia with poor attention span became apparent. The patient improved slowly over 6 months of rehabilitation. Bilateral thalamic lesions can be caused by several conditions. Among those are thiamine deficiency, cerebral lupus, toxoplasmosis, cysticercosis, cerebral syphilitic gumma, and even tumors and fungal infections. All these were ruled out in our case. Superselective digital subtraction angiography (DSA) demonstrated a single unpaired thalamic perforator. To our knowledge, this is the first time this anatomical variant has been demonstrated in vivo in association with bilateral thalamic infarction.
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PMID:Bilateral paramedian thalamic infarct in the presence of an unpaired thalamic perforating artery. 1195 45

Lupus Anticoagulants (LA) pathology is a disease frequently associated with venous and/or arterial thrombosis. The authors describe a 6 year-old boy complaining cephalea and emesis, followed by lethargy, in whom antiphospholipids antibodies were detected. Brain magnetic resonance scans showed lesions of the cerebellum. The characteristics of LA disease are discussed.
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PMID:Infantile cerebellar thrombosis: a case of lupus anticoagulants? 1249 44

To describe the clinical and immunologic characteristics of patients with adrenal involvement and antiphospholipid syndrome (APS), we conducted a computer-assisted (PubMed) search of the literature to identify all cases of primary adrenal insufficiency associated with antiphospholipid antibodies published in English, French, and Spanish from 1983 (when APS was first defined) through March 2002. We reviewed 86 patients (80 from the literature plus 6 from our cohort); 55% were male, and the mean age at presentation was 43 +/- 16 years. Sixty-one (71%) patients had primary APS, and 14 (16%) had systemic lupus erythematosus. In 31 (36%) patients, adrenal insufficiency was the first clinical manifestation of APS. Abdominal pain was present in 55% of patients, followed by hypotension (54%), fever (40%), nausea or vomiting (31%), weakness or fatigue (31%), and lethargy or altered mental status (19%). The main finding in imaging techniques was compatible with adrenal hemorrhage (59%) and in histopathologic study was a hemorrhagic infarction with vessel thrombosis (55%). Lupus anticoagulant was detected in 97% of patients and the anticardiolipin antibodies titer was positive in 93% of patients. Most patients (95%) were positive for the IgG isotype of anticardiolipin antibodies, whereas 40% were positive for the IgM isotype. Baseline cortisol levels were decreased in 98% of patients, ACTH hormone levels were increased in 96% of patients, and the cosyntropin stimulation test was positive in 100% of patients tested. Steroid replacement therapy was the most frequent treatment (84%), followed by anticoagulation (52%) and aspirin (6%). Thirty-two of 35 (91%) patients with prolonged anticoagulant therapy were in good health with a mean follow-up of 25 months, whereas 25 of the 69 (36%) patients with outcome data available had died. The results of the present review stress the clinical importance of systematic screening for lupus anticoagulant and anticardiolipin antibodies in all cases of adrenal hemorrhage or infarction. An initial screening for hypoadrenalism is mandatory in any antiphospholipid antibody-positive patient who complains of abdominal pain and undue weakness or asthenia.
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PMID:Adrenal involvement in the antiphospholipid syndrome: clinical and immunologic characteristics of 86 patients. 1264 Jan 87

A 5-year-old Poodle-cross was initially presented for exercise intolerance and difficulty in chewing and yawning. Some months later it acutely developed lethargy referable to complete heart block. Further investigations before and after permanent pacemaker implantation demonstrated Coombs-positive immune-mediated haemolytic anaemia, presumptive masticatory myositis and hypoadrenocorticism, suggesting the possibility of multisystem auto-immune disease. A diagnosis of systemic lupus erythematosus (SLE) was made based on these findings and a positive anti-nuclear antibody titre. It was thought that immune-mediated destruction of cardiac conduction tissues was responsible for the development of atrioventricular conduction block. Glucocorticoid deficiency was corrected using cortisone replacement therapy. SLE was controlled successfully for 10 months using azathioprine monotherapy until signs, subsequently shown to be due to subacute bacterial endocarditis, resulted in the death of the patient. Lupus should be considered as a potential underlying aetiology in dogs that develop heart block.
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PMID:Complete heart block associated with lupus in a dog. 1508 50

Central nervous system symptoms occur in a substantial portion of patients with systemic lupus erythematosus. However, coma is a rare presentation and is usually secondary to complications such as subarachnoid hemorrhage, seizure, or ischemia. Here, we present a 49-year-old woman with lupus erythematosus and a history of recurrent aseptic meningitis and mild subarachnoid hemorrhage who presented with altered mental status and lethargy that progressed rapidly over hours to the herniation syndrome of coma, extensor posturing, and unilateral pupillary dilation. Spinal fluid showed massive protein elevation (>1600), and head computed tomography revealed global cerebral edema. The clinical and radiologic findings rapidly reversed with intravenous corticosteroids and mannitol within 24 hours, and her mental status improved to baseline. Her course was complicated by 2 episodes of recurrent encephalopathy when corticosteroids were tapered; these resolved after resuming high dosing. Because of ongoing pancytopenia, chemotherapy immunosuppression was delayed, and instead she received intravenous immunoglobulin with improvement in the pancytopenia. She remained cognitively intact during subsequent corticosteroid tapering. Rapid development of coma in lupus patients may be due to a primary process of the disease impacting blood brain barrier integrity. Although rare, this potentially fatal complication may be reversible with acute corticosteroid administration.
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PMID:Transient life-threatening cerebral edema in a patient with systemic lupus erythematosus. 1945 59


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