Repair of an inguinoscrotal hernia in a patient with Becker muscular dystrophy.

INTRODUCTION: Inguinal hernia repairs are routinely performed as outpatient procedures in most patients, whereas a few require admission due to clinical or social peculiarities. Muscular dystrophies are inherited disorders characterized by progressive muscle wasting and weakness. In case of surgery there is no definite recommendation for either general or regional anesthesia. CASE REPORT: This contribution regards a 48 y. o. male patient diagnosed with Becker Muscular Dystrophy by muscle biopsy 10 years earlier. He had a left-sided sizable inguinoscrotal hernia with repeat episodes of incarceration. An elective mesh repair with suction drainage was accomplished under selective spinal anesthesia. The post-operative course was uneventful. DISCUSSION: A few inguinal hernia repairs require admission due to peculiarities such as extensive scrotal hernias requiring suction drainage. Muscular dystrophies are inherited disorders with no cure and no two dystrophy patients are exactly alike, therefore the health issues will be different for each individual. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards the successful elective mesh repair with suction drainage of a large left-sided inguino-scrotal hernia in a 48 y. o. male patient affected by Becker muscular dystrophy by selective spinal anesthesia obtained by 10 milligrams of hyperbaric bupivacaine. CONCLUSION: Effective mesh repair with suction drainage of large inguinal hernias under spinal anesthesia can be achieved in patients affected by muscular dystrophy.

Impaired fibrinolysis in obstructive jaundice--evidence from clinical and experimental studies.

Microvascular thrombosis is considered an important pathogenetic factor in renal failure associated with obstructive jaundice but the mechanisms leading to fibrin deposition are still unknown. The plasma levels of plasminogen activator inhibitor (PAI) in 29 patients with obstructive jaundice were found significantly increased as compared to 20 nonjaundiced patients. Fibrin autography of plasma supplemented with tissue plasminogen activator (t-PA) revealed that in icteric samples most of the added activator migrated with an apparent Mr of 100 kDa, corresponding to t-PA-PAI complex, whereas in control samples virtually all t-PA migrated as free enzyme. PAI activity detected in icteric samples is similar to the endothelial type PAI since it is neutralized by a monoclonal antibody against PAI-1. Venous stasis in jaundiced patients was neither associated with an increase in blood fibrinolytic activity nor with a decrease in PAI activity. Immunologic assay showed that t-PA release was impaired in 3 out of 4 patients. In controls, venous occlusion induced an increase in both fibrinolytic activity and t-PA antigen and a reduction in PAI activity. Bile duct recanalization in jaundiced patients subjected to surgery was accompanied by a decrease in plasma PAI activity which paralleled the decrease in serum bilirubin levels. In nonjaundiced patients, surgical treatment did not cause significant changes in either parameter. Rabbits made icteric by bile duct ligation showed an early and progressive increase in plasma PAI activity indicating that obstructive jaundice itself causes the elevation of circulating PAI. it is concluded that obstructive jaundice is associated with a severe impairment of fibrinolysis which might contribute to microvascular thrombosis and renal failure.

Mucocutaneous paraneoplastic syndromes (MCPS). A clinical-pathologic review.

Mucocutaneous paraneoplastic syndromes (MCPS) are a common group of dermatoses exhibiting a variable morphologic and pathologic picture which can occur in association with solid tumors or hematologic malignancies. In this review the Authors report the most important clinical characteristics and commonly associated malignancies of these dermatoses. Their identification is important because their superficial appearance and precocious arising are useful in the early diagnosis of an otherwise asymptomatic visceral malignancy and because differential diagnosis between skin metastases and malignancy-associated dermatoses is very important for a careful staging and management of the neoplasm. At the same time, MCPS may also be suggestive of the specific type of cancer present. Finally, the presence of a MCPS often carries grave oncologic implications. Once, therefore, the diagnosis of these dermatoses has been established, either an appropriate evaluation for an asymptomatic neoplasm in a cancer-free individual or an investigation for the recurrence of malignancy in an oncologic patient should be initiated.

The design, fabrication and evaluation of a trileaflet prosthetic heart valve.

Although prosthetic heart valves have been in existence for many years, the need for new improved designs and in-vitro evaluation techniques are apparent. This paper presents details on the design considerations, fabrication techniques and heart valve evaluation equipment. A valve performance index is discussed in light of various valve and mock circulatory test section designs. The need for national and indeed international valve evaluation techniques is made apparent.

Increased procoagulant activity of peripheral blood monocytes in human and experimental obstructive jaundice.

We studied the procoagulant activity of peripheral blood monocytes in 41 patients with severe obstructive jaundice and in 27 nonjaundiced control patients using a one-stage clotting assay. Mononuclear cells from jaundiced patients, tested immediately after isolation, expressed low levels of procoagulant activity, which were, however, significantly higher than in cells from controls (p less than 0.01). In addition, after incubation in short-term cultures with and without endotoxin, these cells generated more procoagulant activity than did the control ones (p less than 0.001). No significant difference in procoagulant activity was found between patients with and without malignancy in either group. The relief of biliary obstruction resulted in the reduction of both serum bilirubin levels and monocyte procoagulant activity. Endotoxin-induced monocyte procoagulant activity was about threefold higher in the jaundiced patients who died than in the survivors (p less than 0.001). In rabbits made icteric by bile duct ligation and separation (15 days), the endotoxin-induced monocyte procoagulant activity was markedly increased as compared with sham-operated animals (p less than 0.005). In all instances, procoagulant activity was identified as tissue factor. The increased capacity of mononuclear phagocytes to produce procoagulant activity might help explain the activation of blood coagulation in severe obstructive jaundice.

Increased in vitro and in vivo generation of procoagulant activity (tissue factor) by mononuclear phagocytes after intralipid infusion in rabbits.

Intralipid, a fat emulsion widely used in parenteral nutrition, can produce marked functional changes of the mononuclear phagocyte system. We investigated the effect of Intralipid administration on the generation of procoagulant activity by rabbit mononuclear phagocytes. Two groups of ten rabbits given either a single infusion of Intralipid 10% or a similar volume of sterile saline were studied before and after infusion. Procoagulant activity was measured on isolated blood mononuclear cells after incubation with and without endotoxin, using a one-stage clotting assay. Cells from animals infused with Intralipid produced significantly more procoagulant activity than controls (P less than .01). Results were similar when freshly collected whole blood was incubated with and without endotoxin, and procoagulant activity was measured on subsequently isolated mononuclear cells (P less than .01). In addition, when rabbits were given a single injection of endotoxin, blood and spleen mononuclear cells harvested 50 to 60 minutes after the injection from animals pretreated with Intralipid expressed five to seven times more procoagulant activity than did cells from animals pretreated with saline. In all instances, procoagulant activity was identified as tissue factor. These findings suggest that Intralipid may cause functional changes in mononuclear phagocytes, resulting in increased production of tissue factor on incubation in short-term culture in vitro and in response to endotoxin in vivo.