Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report this rare case of fatal fulminant sepsis in a 42-year-old African American female who presented with a three-day history of generalized pain and an evolving rash all over her body. On presentation, the patient was tachycardic, borderline hypotensive, and febrile. Physical examination was significant for diffuse petechiae and ecchymoses over the extremities, torso, and the face, especially confluent over her thighs and lower abdomen. She was admitted to the ICU, and initial investigations revealed a normal leukocyte count and hemoglobin but severe thrombocytopenia, elevated creatinine, blood urea nitrogen (BUN), bilirubin, transaminases, and an elevated INR. She also had a high anion gap metabolic acidosis with elevated lactate. Chest and abdomen CT findings were nonspecific, demonstrating fluid surrounding both kidneys, a moderate amount of fluid in the pelvis, and alveolar opacities at the bases of both lungs. Initial working diagnoses were a septic shock, thrombotic thrombocytopenic purpura (TTP), and vasculitis. She was initiated on broad-spectrum antibiotic coverage with vancomycin, piperacillin/tazobactam, and doxycycline pending culture reports. After a few hours, she became progressively hypothermic, developed disseminated intravascular coagulation (DIC) and hemodynamic instability, and was intubated due to acute hypoxic and hypercapnic respiratory failure. She progressively worsened hemodynamically with multi-organ dysfunction, and ultimately was pronounced dead roughly 18 hours after initial presentation. Blood cultures grew a Gram-negative organism, initially reported as Shewanella putrefaciens, but subsequently confirmed as Capnocytophaga canimorsus.
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PMID:Overwhelming Sepsis due to Capnocytophaga canimorsus in an Immunocompetent Individual: A Rare Case Study. 3302 57

Pauci-immune crescentic glomerulonephritis is the most common variant of rapidly progressive glomerulonephritis, accounting for approximately 80% of total cases. Most of the cases are associated with the presence of anti-neutrophil cytoplasmic antibody (ANCA) and are usually referred to as ANCA-associated vasculitis. A 68-year-old male with no previous renal history presented with complaints of shortness of breath, cough, and bilateral leg swelling for 2 weeks. Initial workup was significant for creatinine elevated at 2.9 mg/dL, blood urea nitrogen at 65 mg/dL, and glomerular filtration rate of 27 mL/min. Further workup was unremarkable for any significant abnormality. Subsequently patient's kidney function worsened, and temporary hemodialysis was started. Kidney biopsy was performed, which later came back significant for necrotizing arteritis, multifocal, with focal necrotizing and crescentic glomerulonephritis, pauci-immune type. High-dose corticosteroids were administered, and good clinical response was noticed. This is a very rare case of renal limited pauci-immune crescentic glomerulonephritis with annual incidence of 7 to 10 cases per million every year in the United States. The absence of involvement of other organs makes our case even rarer. Mortality is as high as 90% in untreated patients and aggressive therapy with glucocorticoids and cyclophosphamide or rituximab are the mainstay of treatment. The presence of significant renal impairment in the absence of other organs involvement in our patient makes it a very unique presentation of ANCA-positive vasculitis.
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PMID:Renal limited ANCA-positive vasculitis: a rare manifestation of a rare disease. 3323 33


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