Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
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Hereditary hemorrhagic telangiectasia, commonly known as Osler-Weber-Rendu disease, is a systemic autosomal dominant inherited disorder, that occurs in Caucasian populations. We report the case of a 56-year-old housewife who was admitted to the gastrointestinal and cardiovascular ward because she had suffered from recurrent gastrointestinal bleeding and heart failure from 1994 to 1997. Panendoscopy showed vascular ectasia scattered over the tongue, larynx, esophagus, and posterior wall of the gastric body. Colonoscopy showed clusters of telangiectasia over the cecum and ascending colon. Arteriovenous malformations (AVMs) were found in the liver and lungs on computerized tomography. Recurrent gastrointestinal bleeding was controlled by estrogen treatment during the follow-up period. In July 1997, the patient was readmitted to our cardiovascular section due to aggravated dyspnea, orthopnea and bilateral lower leg edema. Cardiac catheterization showed a large fistula from the left pulmonary artery to the left atrium and left ventricle, pulmonary arterial pressure of 37/13 mmHg and cardiac output of 9.61/minute. Other studies excluded the possibility of sepsis, and high-output cardiac failure was suspected. The patient was discharged in a stable condition and scheduled for AVM embolization management. Unfortunately, she died of a suspected heart attack at home two weeks following discharge.
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PMID:Recurrent gastrointestinal bleeding and high output cardiac failure caused by hereditary hemorrhagic telangiectasia. 1082 Sep 15

Bronchiolitis obliterans organizing pneumonia (BOOP) has been described after bone marrow, lung, heart-lung, and renal transplantation, but rarely after orthotopic liver transplantation (OLT). We report a case of BOOP after OLT to emphasize BOOP as an under diagnosed and treatable cause of nonresolving pneumonia, which may not be preventable by maintenance low-dose prednisone. A 48-year-old man was hospitalized for dyspnea and cough one month after OLT. Among his medications were tacrolimus and prednisone. Physical examination was significant for lung crepitations and bilateral leg edema. Chest x-ray revealed bilateral infiltrates. Computed tomography (CT) of the chest demonstrated bilateral diffuse infiltrates with areas of sparing and nodularities. Bronchoscopy was normal and bronchoalveolar lavage was negative. Lung biopsy was performed and demonstrated serpiginous plugs of fibroblastic tissue filling the alveolar spaces, focal fibrosis of some alveolar septa, and reactive pneumocytic hyperplasia consistent with BOOP. Methylprednisolone was continued with clinical improvement and weaning from the ventilator, but subsequent sepsis and multisystem organ failure finally led to the patient's death.
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PMID:Bronchiolitis obliterans organizing pneumonia after orthotopic liver transplantation. 1500 77

We report on a young pregnant woman developing distal leg edema and hypoalbuminemia, who was lately diagnosed with AL amyloidosis. Fetal growth retardation led to a caesarian section in the 27th week of gestation. A live birth healthy female, 710 g weight, was admitted to the neonatal intensive care unit and survived. Thereafter the mother underwent specific chemotherapy achieving only a partial and transient response, and eventually died due to sepsis. Interestingly, amyloidotic material was found on the maternal but not on the fetal side of the placenta. Experimental data show suppression of AA amyloid formation during pregnancy and suggest a protective role of the placenta on the offspring. However, most reported cases deal with pregnant women diagnosed with AA amyloidosis associated with Familial Mediterranean Fever and describe growth retardation of the fetus, worsening renal function and preeclampsia. To the best of our knowledge, this is the first report of AL amyloidosis diagnosed in a pregnant woman. In our patient, as well as in the other reported cases, amyloidosis during pregnancy has been confirmed to be an ominous condition. Therefore mild leg edema and proteinuria during pregnancy, though a common finding, may not be innocent.
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PMID:How nature preserves fetuses. 2323 36

Purulent pericarditis is a rare disease in the antibiotic era. The common pathogens of purulent pericarditis are gram-positive species such as Staphylococcus aureus. Streptococcus pneumoniae, Salmonella, Haemophilus, fungal pathogens/tuberculosis can also result in purulent pericarditis. We report an old male case of purulent pericarditis by Escherichia coli. He came to our hospital suffering from leg edema for 3 months. Echocardiography revealed the large amount of pericardial effusion, and he was admitted to test the cause of pericardial effusion without high fever, tachycardia, and shock vital signs. On the third day, he suddenly presented vital shock. We performed emergency cardiopulmonary resuscitation and pericardiocentesis. Appearance of pericardial effusion was hemorrhagic and purulent. The gram stain revealed remarkable E. coli invasion to pericardial space. Antibiotic therapy was immediately started; however, he died on sixth day with septic shock. The cytological examination of pericardial effusion suggested the invasion of malignant lymphoma to pericardium. This case showed subacute or chronic process of pericarditis without severe clinical and laboratory sings before admission. Nevertheless, bacterial purulent pericarditis usually shows acute clinical manifestation; the first process of this case was very silent. Immunosuppression of malignant lymphoma might make E. coli translocation from gastrointestinal tract to pericardial space, and bacterial pericarditis was progressed to purulent pericarditis. In the latter process, this case showed unexpected rush progression to death by sepsis from purulent pericarditis. Immediate pericardiocentesis should be performed for a prompt diagnosis of purulent pericarditis, and it might have improved the outcome of this case.
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PMID:A Rare Case of Rush Progression of Purulent Pericarditis by Escherichia coli in a Patient with Malignant Lymphoma. 2962 70