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Biomedical subjects

W B Smith

Publications and source records attributed to W B Smith.

At least 73 records · Page 4Linked to original sources

Subdural electrodes for seizure focus localization.

Fifty patients with medically refractory partial seizure disorders have undergone subdural electrode placement for seizure focus localization. Standard scalp telemetry recordings of ictal events had failed to demonstrate accurately the site of seizure onset, and these patients were considered candidates for telemetry with intracerebral depth electrodes. Excellent recordings of interictal and ictal events were obtained, and localization of the epileptogenic focus was derived from recordings made during spontaneously occurring seizures. Electrocorticograms were monitored for up to 21 days. The recordings enabled a surgical decision to be made in 43 of 50 cases (86%). Thirty patients have subsequently undergone cortical excision of their foci with good results. Subdural electrode recordings are a significant addition to the armamentarium of the neurosurgeon attempting to localize surgical seizure foci, offering a low morbidity procedure as an alternative to depth electrode implantation.

Adolescent↗

Elective cardiac operation in a patient with severe hemophilia and acquired factor VIII antibodies.

This paper describes a successful cardiac operation in a young boy with hemophilia, congenital heart disease, severe factor VIII deficiency, and an acquired high titer antibody to factor VIII. To our knowledge, there have been no published cases of elective cardiac operations in a person with severe hemophilia and an accompanying complex problem. Utilizing the team approach, we administered a megadose bolus of factor VIII concentrate preoperatively (eight times the calculated dose), followed by a continuous intravenous infusion at 500 units/hr throughout the procedure and at a reduced dose for the first 5 postoperative days. With the anamnestic rise in factor VIII antibody on day 5, activated prothrombin complex concentrates were substituted for factor VIII and provided continued adequate hemostasis during the remaining 9 postoperative days. The rapid infusion of large quantities of factor VIII was effective in neutralizing the low titer inhibitor and providing normal hemostasis during the procedure. In addition, activated prothrombin complex concentrates were substituted for factor VIII coagulant without recurrent bleeding or thromboembolic phenomena.

Antibodies↗

Head trauma in hemophilia. A prospective study.

Intracranial hemorrhage (ICH) remains a serious problem in hemophilia. Consecutive episodes of ICH or reports of head trauma were prospectively recorded in 140 patients with hemophilia seen during a two-year period. Forty of 48 consecutive episodes of ICH or trauma occurred in 37 patients with severe disease (less than 1% factor VIII or IX). Five injured patients had inhibitors to factor VIII. Two fatalities occurred following injury and one patient died following a spontaneous bleed. Injury in 40 instances warranted replacement therapy. No patient given coagulation factor within six hours of injury experienced ICH. The risk of ICH following head trauma (13% in this series) warrants aggressive use of coagulation factor replacement. Watchfulness in dealing with such patients and early therapy may be more necessary than often realized.

Adolescent↗

Hb S Mobile: a new genetic combination.

The genetic combination of Hb Mobile (beta 73 Asp replaced by Val) and Hb S (beta 6 Glu replaced by Val) was found in a healthy black man whose hemolysate resembles that of Hb S electrophoretically. His mother and sister have Hb AS; the father and brother have Hb A Mobile. No clinical nor hematologic abnormalities were detected in any member of the family. A slightly decreased oxygen affinity associated with Hb Mobile appears to have no clinical significance. Simple methods of differentiating Hb Mobile S from the electrophoretically similar but clinically severe Hb SD Los Angeles are described.

Adolescent↗

Electron spin resonance and fluorescence observations on erythrocytes, erythrocyte membranes, 13762 MAT-A ascites adenocarcinoma cells, and their membranes, effects of membrane perturbations.

Membranes from erythrocytes or MAT-A 13762 tumor cells were labeled with the fatty acid spin probe I(5,10) or ANS and examined by spin resonance (ESR) or fluorescence polarization in the presence or absence of the perturbants EDTA, trypsin, glutaraldehyde, and dodecylsulfate. Extraction of cell membranes with hypotonic EDTA produced fragments in which the order parameters and fluorescence polarization values increased. Fluorescence polarization values using membranes labeled with diphenylhexatriene showed an apparent increase in membrane fluidity. A large portion of both I(5,10) and both fluorescence probes coextract with the peripheral membrane proteins in both membrane systems. Paramagnetic quenching of tryptophan fluorescence with I(5,10) and the spectral characteristics of ANS in these membranes indicated further that significant amounts of both probes bind either at or near the protein-lipid interface or directly to protein moieties. Trypsinization of cell membranes, which preferentially cleaves the large cytoskeletal proteins, fragmented the membranes and reduced the ESR order parameter. Glutaraldehyde immobilized I(5,10) in both types of membranes. These studies suggest that the association of cytoskeletal proteins with the membrane does not have any pronounced, consistent effect on biophysical properties of the bilayer. Attempts to apply these same probes to studies of the plasma membranes of intact cells were not successful because of the diffusion of the probes into the cells. These studies also point out some difficulties in using probe-group techniques to determine the nature of changes in bilayer structural parameters and emphasize the need for a better understanding of probe-group localization and behavior in such studies.

Adenocarcinoma↗

Micromyoclonic seizures of sleep.

A patient with primary generalized epilepsy had convulsive seizures refractory to anticonvulsant therapy. Several waking EEGs failed to show paroxysmal activity, but a sleep recording revealed polyspike EEG discharges associated with individual electromyographic discharges localized to the chin. The discharges occurred with a 90- to 100-minute period during the night. An increased frequency and duration of the EEG discharges on the last night of recording preceded a marked increase in the number of convulsions the following day.

Adult↗

Histiocytosis X: clinical trial of chlorambucil: a report from Childrens Cancer Study Group.

A prospective study for histiocytosis X was designed to determine whether "good risk" patients, ie, those without evidence of dysfunction of liver, lung, or hemopoietic system, would respond to single agent therapy; in this case chlorambucil (CMB) used in a dose of 5 mgm/m2/day. If there was no response after an adequate trial period, treatment was initiated with four drugs using a combination of prednisone, vinblastine, cyclophosphamide and methotrexate. There were 26 evaluable patients, 57% of whom were less than two years of age at onset of therapy. There were three complete and four partial responses to CMB for a response rate of 26.9%. Sixteen patients received an adequate trial of four-drug therapy with three complete and two partial responses for a response rate of 33%. These responses were inferior to those previously reported for either single agents or combined therapy in histiocytosis X.

Adolescent↗

Angiokeratomas, connective tissue nevus, hemangioma.

A 7-year-old white boy had multiple angiokeratomas, connective tissue nevus of the right foot, and cavernous hemangioma with hemiatrophy of the right lower extremity. The triad of abnormalities is distinct from previously described multiple malformation syndromes.

Angiokeratoma↗

Polyvalent pneumococcal-polysaccharide immunization of patients with sickle-cell anemia and patients with splenectomy.

To reduce the risk of infection from Streptococcus pneumoniae in hyposplenic patients we administered octavalent pneumococcal vaccine to 77 patients with sickle-cell disease and 19 asplenic persons and compared their response with 82 controls (38 age-matched normal persons and 44 normal black African children). Fifty micrograms each of pneumococcal-polysaccharide Types 1, 3, 6, 7, 14, 18, 19, and 23 were administered subcutaneously. Post-immunization serums (three to four weeks) were available from 52 of 77 patients with sickle-cell disease; the percent responding and the magnitude of the indirect hemagglutination response were comparable to those of the controls. Within two years after immunization we observed eight Str. pneumoniae infections in 106 age-matched unimmunized patients with sickle-cell disease, but none in the 77 immunized (P less than 0.025). We conclude that pneumococcal polysaccharides are immunogenic in hyposplenic patients and may protect against systemic Str. pneumoniae infection.

Adolescent↗