"The U.S. Food and Drug Administration today approved Vimizim (elosulfase alfa), the first FDA-approved treatment for Mucopolysaccharidosis Type IVA (Morquio A syndrome). Morquio A syndrome is a rare, autosomal recessive lysosomal storage disease "...
(Generic versions may still be available.)
Progressive widening of the QRS complex, prolonged Q-T and P-R intervals, lowering of the R and T waves, as well as increasing A-V block, may be seen with cases which are excessive for a given patient. Increased ventricular extrasystoles, or even ventricular tachycardia or fibrillation may occur. After intravenous administration but seldom after oral therapy, transient high plasma levels of PA may induce hypotension, affecting systolic more than diastolic pressures, especially in hypertensive patients. Such high levels may also produce central nervous depression, tremor, and even respiratory depression.
Plasma levels above 10 µg/mL are increasingly associated with toxic findings, which are seen occasionally in the 10 to 12 µg/mL range, more often in the 12 to 15 µg/mL range, and commonly in patients with plasma levels greater than 15 µg/mL. A single oral dose of 2 g may produce overdosage symptoms, while 3g may be dangerous, especially if the patient is a slow acetylator, has decreased renal function, or underlying organic heart disease.
Treatment of overdosage or toxic manifestations includes general supportive measures, close observation, monitoring of vital signs and possibly intravenous pressor agents and mechanical cardiorespiratory support. If available, PA and NAPA plasma levels may be helpful in assessing the potential degree of toxicity and response to therapy. Both PA and NAPA are removed from the circulation by hemodialysis but not peritoneal dialysis. No specific antidote for PA is known.
Complete Heart Block: Procainamide should not be administered to patients with complete heart block because of its effects in suppressing nodal or ventricular pacemakers and the hazard of asystole. It may be difficult to recognize complete heart block in patients with ventricular tachycardia, but if significant slowing of ventricular rate occurs during PA treatment without evidence of A-V conduction appearing, PA should be stopped. In cases of second degree A-V block or various types of hemiblock, PA should be avoided or discontinued because of the possibility of increased severity of block, unless the ventricular rate is controlled by an electrical pacemaker.
Idiosyncratic Hypersensitivity: In patients sensitive to procaine or other ester-type local anesthetics , cross sensitivity to PA is unlikely; however, it should be borne in mind, and PA should not be used if it produces acute allergic dermatitis, asthma, or anaphylactic symptoms,
Torsades de Pointes: In the unusual ventricular arrhythmia called "les torsades de pointes" (twisting of the points) characterized by alternation of one or more ventricular premature beats in the directions of QRS complexes on EGG in persons with prolonged Q-T and often enhanced U waves, Group 1 A antiarrhythmic drugs are contraindicated. Administration of PA in such cases may aggravate this special type of ventricular extrasystole or tachycardia instead of suppressing it.
Last reviewed on RxList: 12/8/2004
This monograph has been modified to include the generic and brand name in many instances.
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