Melricflash/CW_MedAbstractsAlt huggingface dataset:
DNA sequence analysis of three inhibitor-positive hemophilia B patients without gross gene deletion: identification of four novel mutations in factor IX gene. Three hemophilia B patients with anti-factor IX antibodies who had no detectable gross deletion of the factor IX gene by Southern blotting analysis were investigated at the molecular level. All eight exons, accompanied by their splicing junction sites and presumptive promoter regions of the factor IX gene in these patients (total 5.5 kb in length) were amplified with the use of the polymerase chain reaction, followed by complete nucleotide sequence analysis. Three different types of novel single base substitutions and a 2 base-pair nucleotide deletion were identified. Patient HB-5 had two point mutations in his factor IX gene. One was located at the promoter region at nucleotide -793 and the other (C-to-T transition) was found in exon VI of the gene changing Gln-191 to a stop codon. Patient HB-6 had a point mutation (G-to-A) in the splice acceptor site, which interrupted the normal splicing of the last intron G. A small two-nucleotide deletion in exon III was detected in patient HB-7 and yielded frameshifted amino acids and terminated by a stop codon. These resuslts suggest that not only the gross gene deletion of factor IX gene but also the point mutations or small nucleotide deletion that may cause the interruption of coding informations for mature protein synthesis is predisposed to development of anti-factor IX inhibitors in patients with hemophilia B. 

Category: general pathological conditions
Internal capsule plaque and tonic spasms in multiple sclerosis. A patient developed hemilateral tonic spasms associated with a relapse of multiple sclerosis. An area of demyelination in the right internal capsule was observed on a magnetic resonance imaging scan. This lesion was not detectable on a second magnetic resonance imaging scan 10 months after the spasms had ceased. Paroxysmal symptoms in multiple sclerosis may represent transient phenomena related to inflammation in acute plaques. 

Category: general pathological conditions
Timing of corticosteroid treatment. Effect of lung lymph dynamics in air injury in awake sheep. In paired experiments, we studied the effects of high-dose methylprednisolone on the acute pulmonary injury caused by 4 h of venous air embolization in 19 chronically instrumented, unanesthetized sheep with lung lymph fistulas. We compared the effect of methylprednisolone (30 mg/kg intravenous bolus) given before embolization, early (1 H) in the course of embolization, late (3 h) in the course of embolization, or after embolization (at the beginning of the recovery period). We measured pulmonary hemodynamics and lymph dynamics. In six sheep we also fixed lung tissue for semiquantitative histology, and in some we measured leukocyte concentrations in blood and in pulmonary lymph. Methylprednisolone did not significantly affect pulmonary hemodynamics but it largely prevented lung injury when it was given before embolization. It also lessened the degree of lung injury when it was given during embolization, although this effect became less marked as treatment was delayed. Methylprednisolone had no effect on lung injury when given after embolization was completed (4 h). We found fewer leukocytes attached to air emboli and fewer endothelial cell gaps in the lungs of sheep given methylprednisolone as prophylaxis. Leukocyte counts were lower in lung lymph and higher in the circulating blood of methylprednisolone-treated sheep. We conclude that methylprednisolone has a preventive effect on air embolism lung injury, such that its effect is greater when given earlier during the development of injury. 

Category: cardiovascular diseases
Hospitalization and the cognitive deficits of schizophrenia. The influences of age and education. The study investigated the relationship between length of hospitalization and increasing cognitive deficit in schizophrenics. Using Halstead-Reitan Battery data obtained from 245 schizophrenic patients, multiple regression analyses were performed using age, education, and length of hospitalization as independent variables and various summary test indices as dependent variables. These analyses showed that there was not a statistically significant change in percentage of explained variance when length of hospitalization was entered into the multiple regression equations. On the basis of these analyses, it was concluded that the association between increasing deficit and length of hospitalization experienced by schizophrenic patients is no greater than what would be anticipated on the basis of aging. 

Category: nervous system diseases
Prostacyclin but not phentolamine increases oxygen consumption and skin microvascular blood flow in patients with sepsis and respiratory failure. Inadequate tissue oxygenation may occur in critically ill patients with sepsis despite an apparently adequate O2 transport (QO2), and this may contribute to the development of an O2 debt and also to multiple organ failure. It has been shown that increasing QO2 by infusing a vasodilator may reveal this O2 debt in septic patients. To investigate whether the site of action of vasodilators may be of importance in unmasking such an O2 debt, we administered prostacyclin, a prostaglandin with a preferential effect on the microcirculation, and phentolamine, an arteriolar vasodilator, in 11 patients studied during the first 48 hours after the onset of sepsis, and compared their effect on whole body oxygen consumption (VO2) and skin microvascular blood flow. The results demonstrated that increasing QO2 by prostacyclin but not by phentolamine significantly increases VO2 in critically ill patients with sepsis. The site of action of vasodilators may therefore play an important role in their ability to unmask an O2 debt. 

Category: general pathological conditions
Autograft versus allograft for benign lesions in children. Benign bone lesions in children are often so large in size that there is not an adequate amount of bone available for an autograft to fill the resultant cavity after surgical curettage. This study compared autografts and allografts with respect to the time required and the success of graft incorporation. Fifty-four patients with 61 lesions were studied. Lesions were classified as small volume (less than 60 cc) or large volume (more than 60 cc) and were separated into four groups: small-volume autograft, large-volume autograft, small-volume allograft, and large-volume allograft. Allografts appeared comparable to autografts when small-volume lesions were treated. The healing time was slightly longer for allografts with an average period of 21 months versus 27 months for autografts. Autografts were superior to allografts in rate and completeness of healing for solitary large lesions. This increased efficacy presumes a somewhat older child in which an adequate amount of bone is available for an autograft. A young child with multicentric or polyostotic lesions can still achieve successful incorporation with allografts. In this study, 38% healed completely and 29% healed partially. Allografts have a distinct place in the treatment of benign bone lesions in children. 

Category: neoplasms
A paradigm for restenosis based on cell biology: clues for the development of new preventive therapies. Angioplasty causes substantial injury to the coronary artery intima and media that is unrecognizable by angiography. On the basis of a substantial body of research in oncology and wound healing, it is hypothesized that restenosis is a manifestation of the general wound healing response expressed specifically in vascular tissue. The temporal response to injury occurs in three characteristic phases: inflammation, granulation and extracellular matrix remodeling. The specific expression of these phases in the coronary artery leads to intimal hyperplasia at 1 to 4 months. The major milestones in the temporal sequence of restenosis are platelet aggregation, inflammatory cell infiltration, release of growth factors, medial smooth muscle cell modulation and proliferation, proteoglycan deposition and extracellular matrix remodeling. Each step has potential inhibitors that could be used for preventive therapy. Resolution of restenosis, however, probably requires both creation of the largest possible residual lumen and substantial inhibition of intimal hyperplasia. 

Category: cardiovascular diseases
Comparison of ventricular arrhythmia induction with use of an indwelling electrode catheter and a newly inserted catheter. Two methods of serial electrophysiologic testing are in widespread use. Most commonly, the electrode catheter is removed after each study and a new catheter reinserted through the femoral vein for every subsequent test. An alternative method employs an electrode catheter that remains in place during several days of serial testing. Little is known about differences between these two methods with respect to the likelihood of induction of arrhythmia or the frequency of complications. To determine whether inducibility of sustained arrhythmia is altered or if the frequency of complications is unacceptably high with use of an indwelling catheter, a prospective randomized study was conducted in 78 patients. Each patient underwent baseline testing, several days of electropharmacologic testing with an indwelling catheter, a 24 h drug elimination period and placement of a new electrode catheter. Ventricular stimulation studies were then performed in each patient with both the indwelling and new electrode catheters. No differences were found between the indwelling and new catheter tests with respect to induction of arrhythmia, number of extrastimuli required to induce arrhythmia, rate of arrhythmia or requirement for cardioversion. Ventricular pacing thresholds were higher and effective refractory periods were slightly longer when measured with the indwelling catheter. Complications related to the 156 catheter insertions included two that may have been related to the indwelling catheter (one episode of staphylococcal sepsis and one presumed pulmonary embolism) and four that were related to invasive procedures (pneumothorax in all). There were no long-term adverse sequelae of these complications. 

Category: general pathological conditions
Acquired retinal arteriovenous communications in occlusive disease of the carotid artery. Retinal arteriovenous communications proximal to extensive areas of complete vascular closure developed in three patients with occlusive disease of the carotid arteries. In one case, the authors document the development of the arteriovenous communications through successive enlargement of small retinal vessels after progressive signs of retinal ischemia in the form of narrowed retinal arteries, venous dilation and beading, microaneurysms, retinal dot and blot hemorrhages, cotton-wool spots, and capillary nonperfusion. The development of arteriovenous communications in carotid occlusive disease has not been described previously. 

Category: general pathological conditions
Differential light scattering cuvettes for the measurement of thromboemboli in high shear blood flow systems. Newly developed optical scattering cuvettes were constructed as a modification of our existing 1.0 mm and 3.0 mm internal diameter (ID) cuvettes to facilitate the measurement of platelet microemboli ranging from 20 microns to 1,000 microns diameter in whole blood in 0.9 mm ID flows ranging from 250 to 4,000 ml/min. A perturbation solution to the one-speed radiative transport equation was used in the design and calibration of these cuvettes. A series of tests were performed with these cuvettes in an extracorporeal left ventricular assist device bovine model, and in a recirculating closed-loop flow system containing anticoagulated whole baboon blood, to determine to what extent they affect platelet and erythrocyte function ex vivo and in vitro. Serial hemolysis tests, thromboxane radioimmunoassay measurements, platelet counts, and activated partial thromboplastin times were measured. All of these tests with cuvettes in the extracorporeal and in vitro circuits were statistically indistinguishable from baseline measurements, suggesting the usefulness of this system for the measurement of microemboli in blood-contacting materials of extracorporeal circuits and cardiac assist devices. 

Category: cardiovascular diseases
Results of radiation therapy in carcinoma of the base of the tongue. The Curie Institute experience with about 166 cases. Between 1960 and 1980, 166 patients with squamous cell carcinoma of the base of the tongue were treated with primary irradiation at the Curie Institute (Paris, France). Distribution according to the TNM system 1978 International Union Against Cancer (UICC) was the following: 22 T1 lesions, 47 T2 lesions, 64 T3 lesions, and 33 T4 lesions. Regional nodes were not palpable in 50 cases, 35 had N1 nodes, 12 had N2 nodes, and 69 had N3 nodes. All patients received external beam radiation. The 2-year, 3-year, and 5-year overall survival rates for all patients were, respectively, 45%, 37%, and 27%. Local control was significantly related to the initial status of the primary, to the tumor regression at the end of the radiation therapy, and to the histologic differentiation. The 2-year local control was 96% for T1 lesions, 57% for T2 lesions, 45% for T3 lesions, and 23% for T4 lesions. Local control was 70% if the tumor regression was complete at the end of the treatment and 27% if the tumor regression was partial. No significant differences were found in primary local control with respect to degree of infiltration, age, and dose of radiation therapy over a dose of 60 Gy in 6 weeks. The 3-year regional control was 86% for N0, 78% for N1, and 60% for N2 and N3. Among the tumor characteristics analyzed, the most useful ones for predicting local control and survival were clinical tumor staging parameters and tumor radiation-induced regression. A new therapeutic approach based on the evaluation of the tumor regression at 50/55 Gy is under discussion. 

Category: neoplasms
Embolism of cerebral tissue: a cause of coagulopathy and cerebral infarction? Report of a case. We report a case of potentially survivable closed head injury, which was complicated by disseminated intravascular coagulation and bilateral cerebral infarction. Autopsy disclosed pulmonary emboli composed of cerebral tissue. We suggest that the entry of brain tissue into the systemic blood circulation caused severe disseminated intravascular coagulation, with consequent thrombosis of cerebral veins and spreading infarction. 

Category: nervous system diseases
Asymptomatic periapical radiolucent lesion found in an area of previous trauma [published erratum appears in J Am Dent Assoc 1991 Feb;122(2):18] The case of an anterior mandibular radiolucent area, which unexpectedly yielded salivary gland tissue, is described. Salivary gland depressions in the posterior mandible are readily recognized because of their almost pathognomonic radiographic appearance. A salivary gland inclusion in the anterior mandible in the proximity of teeth may create a diagnostic dilemma where superimposed periapical pathosis is present. The clinical importance lies in the recognition and appropriate treatment of the lesion. For the anterior salivary gland inclusion, a biopsy and a microscopic examination provide the most reliable diagnosis. 

Category: general pathological conditions
Sudden death due to right ventricular cardiomyopathy. A 21-year-old man died suddenly at a small party. He had had no clinical signs of cardiac disease except for a slightly abnormal electrocardiogram (occasional premature ventricular contractions) since he was 15 years of age. Autopsy examination revealed cardiomegaly (469 g), with right atrial and ventricular dilatation. The right ventricular myocardium was massively replaced with adipose tissue, and there was one isolated fatty lesion in the right side of the ventricular septum. There were no congenital malformations such as a septal defect or valvular deformity. Histologically, muscular fibers remaining in the right ventricular wall showed neither degenerative nor inflammatory changes. An isolated lesion of the ventricular septum consisted of almost complete replacement of the muscle bundles with adipose tissue. Such a pathologic condition has recently been termed right ventricular cardiomyopathy. Postmortem examination is necessary to make a definite diagnosis of the disease, because in most adult cases of the disease, sudden death occurs before there have been any critical signs. 

Category: general pathological conditions
Omeprazole enhances the efficacy of pancreatin (pancrease) in cystic fibrosis. We studied the effect of the addition of omeprazole (20 mg once a day) to treatment with pancreatin (Pancrease, Cilag, Herentals, Belgium), two or four capsules three times a day, on fecal fat excretion in a double-blind, crossover fashion in nine patients with cystic fibrosis having persistent steatorrhea while taking Pancrease, two capsules three times a day (mean fecal fat excretion, 22.3%; range, 12% to 44%). Neither doubling of the dose of Pancrease nor addition of omeprazole to the lower dose of Pancrease significantly reduced fecal fat excretion (mean, 19.6% [range, 10% to 34%]; mean, 16.4% [range, 6% to 32%], respectively). However, addition of omeprazole to the higher dose of Pancrease (four capsules three times a day) significantly reduced fecal fat excretion when compared with the two doses of Pancrease alone (mean, 10.7%; range, 4% to 25%; P less than 0.01). We conclude that adjunct therapy with omeprazole reduces fecal fat excretion in cystic fibrosis provided that a high dose of Pancrease is supplied. 

Category: digestive system diseases
Is local recurrence of minor importance for metastases in soft tissue sarcoma? The authors analyzed the relationship between treatment, local recurrence, and metastases in a population-based series of 375 patients with soft tissue sarcoma of the extremities and the trunk wall. Treatment was inadequate (marginal excision alone) in 107 patients, local recurrence occurred in 112, and 128 patients developed metastases. Local recurrence was 3.5 times more common after inadequate treatment than after adequate and 2.5 times more common in patients with metastases than in those without. However, metastases were only 1.2 times more common after inadequate treatment than after adequate. Of the 128 patients who developed metastases, 63 had local recurrence and 65 had not. In these two subgroups the timing of metastases and the distribution of clinicopathologic factors--age, sex, tumor size, localization, depth, histotype, and malignancy grade--were similar. These findings indicate that local recurrence is of minor importance for development of metastases in soft tissue sarcoma. The increased local recurrence rate in metastatic tumors may be an expression of the aggressiveness of the primary tumor; highly malignant tumors combine a potential both for local and distant spread. 

Category: neoplasms
Control of immediate postoperative pain with topical bupivacaine hydrochloride for laparoscopic Falope ring tubal ligation. Conflicting reports exist in the literature on the effectiveness of topical local anesthetic applied to the serosal surface of the fallopian tubes for the control of immediate postoperative pain after mechanical (band or clip) tubal ligation. Sixty-four patients were studied prospectively during outpatient laparoscopic Falope ring tubal ligation using the modified McGill Present Pain Intensity Scale. Patients randomly assigned to four groups received topical bupivacaine hydrochloride on both fallopian tubes, the right tube only, or the left tube only, or received none (controls). A unique study design was incorporated which allowed the untreated fallopian tube to serve as a within-subject control for each patient receiving unilateral treatment. Statistical analysis confirmed significant benefit when both fallopian tubes were treated as compared with no treatment (P less than .05). There was also consistent evidence of decreased immediate postoperative pain perception on the treated side for patients receiving unilateral treatment. The value of topical bupivacaine was demonstrated by both subjective patient response (McGill Pain Scale) and reduced need for pain medication before outpatient discharge. Our data support the value of topical bupivacaine applied to the serosal surface of the fallopian tubes for the reduction of postoperative pain after outpatient laparoscopic mechanical (band or clip) tubal ligation. 

Category: general pathological conditions
Visceral perception in health and functional dyspepsia. Crossover study of gastric distension with placebo and domperidone. The symptoms of functional dyspepsia are still unexplained. To evaluate the possible role of abnormal visceral perception, we studied the symptomatic responses and the pressure variations during progressive gastric distension in 10 female healthy control subjects (mean age 33.6 years) and in 10 female patients with functional dyspepsia (mean age 35.2 years). A rubber balloon was positioned 4 cm below the lower esophageal sphincter (LES) and inflated with progressively larger volumes of air by steps of 50 ml; pressures at the gastric fundus and at the LES were continuously recorded by perfused manometric catheters. Each subject was studied on two separate occasions after randomized double-blind administration of either placebo or 20 mg of domperidone. Symptomatic responses and the manometric data were analyzed at the time of the initial recognition of distension (bloating step) and at the time of reporting pain or up to a maximum of 700 ml of balloon inflation (pain or 700-ml step). On placebo, the volumes of gastric distension were more than two times lower in patients than in control subjects at the bloating step (185 +/- 32 ml vs 470 +/- 40 ml, P = 0.001) and at the pain or 700-ml step (265 +/- 54 ml vs 600 +/- 34 ml, P less than 0.005), while the pressure gradients (pressure at inflation steps minus baseline pressure before beginning inflation) were not statistically different between the two groups. 

Category: general pathological conditions
Increasing the yield of ventricular tachycardia induction: a prospective, randomized comparative study of the standard ventricular stimulation protocol to a short-to-long protocol and a new two-site protocol. Programmed ventricular stimulation with a standard protocol that used up to three extrastimuli was compared prospectively with a short-to-long protocol and a two-site protocol in 77 consecutive patients undergoing electrophysiologic study in an attempt to increase the yield of ventricular tachycardia (VT) induction. The short-to-long protocol uses a train of eight stimuli at a short cycle length and up to two extrastimuli. The two-site protocol is similar to the standard protocol but delivers the last extrastimulus via a second spatially separated right ventricular catheter. Patients were divided into two groups based on indications for study: group 1 included 45 patients with syncope, nonsustained VT, or both, and group 2 included 32 patients with a history of sustained VT, sudden cardiac death, or both. The yield of VT induction with the short-to-long protocol was less than that with the standard protocol. In none of the patients in group 1 in whom the standard protocol results were negative did the short-to-long protocol produce sustained VT. Only two patients, both in group 2, had sustained arrhythmias induced by the short-to-long protocol when the standard protocol results were negative: one had sustained VT induced and one with long QT syndrome had ventricular fibrillation (VF) induced with the short-to-long protocol. However, the short-to-long protocol failed to induce sustained VT in seven patients in whom the standard protocol produced sustained VT. All seven of these patients required three extrastimuli with the standard protocol for induction of VT. 

Category: cardiovascular diseases