Hepatic iron overload in aceruloplasminaemia.

We report the case of a 52 year old male with diabetes mellitus and long standing evidence of hepatic iron excess. Initially considered to have haemochromatosis, this patient was reevaluated when hepatic iron stores were found to be unaffected by a prolonged course of weekly phlebotomy. The development of neurological disease prompted diagnostic consideration of aceruloplasminaemia, which we confirmed by demonstration of a novel frameshift mutation in the ceruloplasmin gene. Our inability to resolve the patient's iron overload by regular phlebotomy is consistent with recent animal studies indicating an essential role for ceruloplasmin in cellular iron efflux. Evaluation of this case underscores the clinical relevance of aceruloplasminaemia in the differential diagnosis of hepatic iron overload and provides insight into the pathogenetic mechanisms of hepatocellular iron storage and efflux.

Diagnosis of a left-sided superior vena cava during placement of a pulmonary artery catheter.

We report a case of a left sided superior vena cava (SVC) that was diagnosed during placement of a pulmonary artery (PA) catheter. After entering the left internal jugular, the PA catheter passed into the left side of the heart, through the aortic valve, and into the aorta. This was an unusual cause of right-to-left shunting and persistent cyanosis in a patient who had undergone two open cardiac procedures, including repair of an atrial septal defect. Cardiac catheterization and echocardiography also failed to reveal the abnormality. The embryology and physiology of a left sided SVC is reviewed, including an historical perspective. A discussion of the variants of the syndrome is included, as is a review of aberrant placement of central venous catheters.

Indications for the use of pacing pulmonary artery catheters in cardiac surgery.

Several varieties of pulmonary artery catheters (PACs) with pacing capabilities are now available. Although specific recommendations for prophylactic perioperative placement of pacemakers have been offered previously, the authors believe that those recommendations warrant further examination, taking into consideration the availability of new pacing modalities. Toward this end, the use of pacing PACs in cardiac surgical patients was prospectively examined. In 600 consecutive adult patients with PACs placed prior to cardiopulmonary bypass (CPB), the cardiac anesthesiologist recorded if a pacing PAC was placed, the indications for placing it, and whether the catheter was used to pace. If a pacing PAC was not chosen, the anesthesiologist indicated whether cardiac pacing was needed prior to CPB. In all patients, the presence and specifics of the following five possible indications were documented: sinus node dysfunction/bradydysrhythmias, atrioventricular heart block, fascicular or bundle branch block, cardiac reoperation, and/or valvular heart disease. PACs with pacing capability were placed in 180 of the 600 patients (30.0%) and were used in 34 of these 180 patients (18.8%). In 4 of 420 patients (0.95%) without pacing PACs, cardiac pacing was needed prior to CPB. The following preoperative diagnoses were significant predictors (P less than .05) for the use or need for pacing catheters: sinus node dysfunction/bradydysrhythmias, a history of transient complete atrioventricular block, aortic stenosis, aortic insufficiency, and reoperation. The majority of adult patients undergoing cardiac surgery do not require the use of a pacing PAC prior to CPB.(ABSTRACT TRUNCATED AT 250 WORDS)

Pancreatic porcine phospholipase A2 catalyzed hydrolysis of phosphatidylcholine in lecithin-bile salt mixed micelles: kinetic studies in a lecithin-sodium cholate system.

Pancreatic porcine phospholipase A2 catalyzed hydrolysis of phosphatidylcholine in bile salt lecithin mixed micelles has been studied, utilizing a series of assay mixtures for which the micellar size, weight, and composition had been experimentally determined. Under these conditions the enzymatic hydrolysis is dependent on the phosphatidylcholine-to-sodium cholate molar ratio within the mixed micelle rather than the bulk concentration of the phospholipid in the mixture: at 5 mM phosphatidylcholine, variation of the NPC/NNaCh ratio from 0.2 to 2.0 increases the enzymatic activity from 82 to 933 mumol/min/mg protein. The initial rates are linear throughout the entire series of assay mixtures, the activity vs micellar concentration curves exhibit saturation behavior, and treatment of the data according to the "surface-as-cofactor" theory provides linear double-reciprocal plots which intersect in one point. The assay system should be applicable for detailed kinetic studies of lipolytic enzymes, including mammalian phospholipases which exhibit rather low activities toward lecithin-Triton X-100 mixed micelles. The system should also provide a convenient basis for mechanistic studies involving the use of inhibitory phospholipid substrate analogs.