Renal Amyloid-Associated (AA) Amyloidosis in a Sickle Cell Patient: A Case Report and Literature Review.

Renal amyloid-associated (AA) amyloidosis is a rare occurrence in sickle cell disease (SCD). Very little literature is available on renal AA amyloidosis in sickle cell disease. Nephrotic range proteinuria is associated with higher mortality among patients with SCD. We present a case of a young reproductive-age African American woman who presented with massive nephrotic range proteinuria. Other more common causes of AA amyloidosis such as immunologic and infectious etiologies were ruled out by history, physical examination, radiologic investigation, and serology. Renal biopsy showed mesangial expansion with Congo red-positive material. Staining for immunoglobulins was negative. Electron microscopy showed nonbranching fibrils. These findings were consistent with AA amyloidosis. This case report adds to the rare findings of renal AA amyloidosis in sickle cell disease. The patient refused any intervention to decrease her Glomerular Filtration Rate (GFR) in the hopes of potentially reversing the disabling proteinuria. We report sickle cell disease presenting with nephrotic syndrome secondary to AA amyloid.

Update on the management of cardiogenic shock.

PURPOSE OF REVIEW: Cardiogenic shock is a life-threatening emergency that occurs frequently with acute coronary syndromes. If rapid myocardial reperfusion following acute myocardial infarction is not obtained, either with thrombolytics or by revascularization, cardiogenic shock frequently develops and the mortality rate is high. This review summarizes recent advances in the pathophysiology, incidence and treatment of cardiogenic shock. Particular attention is given to pharmacologic advances. RECENT FINDINGS: Cardiogenic shock continues to occur in 5-10% of patients who suffer a myocardial infarction and the mortality remains over 50% in most studies. Treatment preference is referral to a cardiac center capable of reperfusion using multiple therapies. While no delay in reperfusion is acceptable, emphasis on implementing supportive treatment such as vasopressors, inotropes, and fluids remains critical. There is a wide variance in treatment standards despite established guidelines. Overall mortality from cardiogenic shock has decreased but the incidence remains unchanged. SUMMARY: Emerging pharmacological interventions designed to counteract the underlying proinflammatory pathophysiologic mechanisms may, in combination with early revascularization, result in improved patient outcomes, but there is no magic bullet on the horizon. Attention to the timeliness of transport and treatment of patients with a focus on revascularization is required for cardiogenic shock patients.

Determination of constituents of Telazol--tiletamine and zolazepam by a gas chromatography/mass spectrometry-based method.

Tiletamine and zolazepam injection (Telazol) is used in veterinary surgical practice to induce short-term anesthesia and also to immobilize wild animals. The present work describes a sensitive method to measure tiletamine and zolazepam concentrations in plasma by means of GC/EI-MS on a 5% phenyl/95% methylpolysiloxane column. A simple liquid extraction procedure with ethyl acetate was used to isolate the two compounds and the same were separated and analyzed by GC/MS without derivatization. A formal validation of the assay demonstrated good accuracy and precision for both tiletamine (98-100.8%; C.V.total < 6.7%) and zolazepam (98.3-103.4; C.V.total < 13.2%). With 500 microl of plasma, the limits of quantification for both tiletamine and zolazepam were found to be 10 ng/ml. Both compounds were stable after three freeze-thaw cycles. The assay was used to analyze plasma samples collected from a pig after intramuscular administration of 10 mg/kg of Telazol. The plasma concentration-time profile of tiletamine and zolazepam from this representative pig is also provided.