shukoor65/lab-rag-model huggingface dataset:
Linear nevus sebaceous syndrome: megalencephaly and heterotopic gray matter. A 3-month-old white male with linear nevus sebaceous syndrome (LNSS), facial deformities, brain malformations, and cortical blindness is presented. Facial deformities included left ear hypertrophy and a left-sided hamartomatous neck mass. Magnetic resonance imaging suggested that a central nervous system (CNS) abnormality, involving gray and white matter, should be linked with LNSS. Magnetic resonance imaging was more useful than computed tomography in demonstrating the full spectrum of CNS anomalies, including unilateral lissencephaly, a paucity of white matter, excessive and heterotopic gray matter, apparent left schizencephaly, and unilateral left colpocephaly (dilation of atrium and posterior horn of lateral ventricle). Postmortem examination revealed left hemisphere megalencephaly with a dilated posterior horn of the lateral ventricle. The impressive gray matter heterotopias likely were the source of the patient's seizures and perhaps the ultimate cause of death. Megalencephaly and other deviations of neuronal migration should be considered as a dramatic component of LNSS.
The neurology of aging: normal versus pathologic change. Mild changes in neurologic function occur with aging but generally do not substantially interfere with everyday activities unless disease intervenes. Not infrequently, older adults experience minor impairment in memory, speed of cognitive processing, sleep, vision and hearing, vibratory sense in the lower extremities, and gait and posture. In general, these changes correspond with age-associated neuroanatomic changes (eg, diminished brain weight), but there is wide variability in the extent to which these changes occur. Often, "normal" neurologic changes are difficult to distinguish from impairment associated with disease.
In vivo and ex vivo magnetic resonance imaging evaluation of early disc degeneration with histopathologic correlation. The in vivo and ex vivo microanatomic appearance of early disc degeneration were identified by magnetic resonance imaging and correlated with their respective histopathologic findings. Five cadaver spines (18 discs) and 25 patient studies (122 discs) all imaged at 1.5 Tesla were studied. Two signs of early degenerative disc disease were found: infolding and the central dot. Infolding of the central fibers of the outer annulus coalesced into a central dot of low signal intensity that was seen on both the ex vivo and in vivo images. Infolding was seen 29 of 122 times, and the central dot was observed 15 to 122 times on the in vivo images. A later form of degenerative disc disease was identified as a separation of the nucleus pulposus from the hyaline cartilage end-plate. This separation was seen as a linear area of either low or high signal intensity on the ex vivo images but only as a band of high signal intensity on the in vivo spin-echo 2,500-msec/80-msec images. Only 7 of 122 in vivo discs showed this separation. Internal herniation of nucleus pulposus into the outer annulus was seen only on the ex vivo images. Early degenerative disc disease may exist before there is loss of disc height or signal intensity on the long time-to-repetition (TR)/time-to-echo (TE) magnetic resonance images.
Identification of p53 gene mutations in bladder cancers and urine samples. Although bladder cancers are very common, little is known about their molecular pathogenesis. In this study, invasive bladder cancers were evaluated for the presence of gene mutations in the p53 suppressor gene. Of 18 tumors evaluated, 11 (61 percent) were found to have genetic alterations of p53. The alterations included ten point mutations resulting in single amino acid substitutions, and one 24-base pair deletion. In all but one case, the mutations were associated with chromosome 17p allelic deletions, leaving the cells with only mutant forms of the p53 gene products. Through the use of the polymerase chain reaction and oligomer-specific hybridization, p53 mutations were identified in 1 to 7 percent of the cells within the urine sediment of each of three patients tested. The p53 mutations are the first genetic alterations demonstrated to occur in a high proportion of primary invasive bladder cancers. Detection of such mutations ex vivo has clinical implications for monitoring individuals whose tumor cells are shed extracorporeally.
Evaluation of combination thrombolytic therapy and timing of cardiac catheterization in acute myocardial infarction. Results of thrombolysis and angioplasty in myocardial infarction--phase 5 randomized trial. TAMI Study Group Recent trials of myocardial reperfusion using single-agent thrombolytic therapy and sequential cardiac catheterization have supported a conservative approach to the patient with acute myocardial infarction. To evaluate combination thrombolytic therapy and the role of a previously untested strategy for the aggressive use of cardiac catheterization, we performed a multicenter clinical trial with a 3 x 2 factorial design in which 575 patients were randomly allocated to one of three drug regimens--tissue-type plasminogen activator (t-PA) (n = 191), urokinase (n = 190), or both (n = 194) - and one of two catheterization strategies--immediate catheterization with angioplasty for failed thrombolysis (n = 287) or deferred predischarge catheterization on days 5-10 (n = 288). Patients with contraindications to thrombolytic therapy, cardiogenic shock, or age of more than 75 years were excluded. Global left ventricular ejection fraction was well preserved and almost identical at predischarge catheterization (54%), regardless of the catheterization or thrombolytic strategy used (p = 0.98). Combination thrombolytic therapy was associated with a less complicated clinical course, most clearly documented by a lower rate of reocclusion (2%) compared with urokinase (7%) and t-PA (12%) (p = 0.04) and a lower rate of recurrent ischemia (25%) compared with urokinase (35%) and t-PA (31%). When a composite clinical end point (e.g., death, stroke, reinfarction, reocclusion, heart failure, or recurrent ischemia) was examined, combination thrombolytic therapy was associated with greater freedom from any adverse event (68%) compared with either single agent (urokinase, 55%; t-PA, 60%) (p = 0.04) and with a less complicated clinical course when the composite clinical end points were ranked according to clinical severity (p = 0.024). Early patency rates were greater with combination therapy, although predischarge patency rates after considering interventions to maintain patency were similar among drug regimens. No difference in bleeding complication rates was observed with any thrombolytic regimen. The aggressive catheterization strategy led to an overall early patency rate of 96% and a predischarge patency rate of 94% compared with a 90% predischarge patency in the conservative strategy (p = 0.065). The aggressive strategy improved regional wall motion in the infarct region (-2.16 SDs/chord) compared with deferred catheterization (-2.49 SDs/chord) (p = 0.004). More patients treated with the aggressive strategy were free from adverse outcomes (67% versus 55% in the conservative strategy, p = 0.004), and the clinical course was less complicated when the adverse outcomes were ranked according to severity (p = 0.016). No significant increase in use of blood products resulted from the aggressive strategy.(ABSTRACT TRUNCATED AT 400 WORDS).
Retained bile duct stones in a patient with Billroth II gastrectomy: extracorporeal shock wave lithotripsy and papillary dilatation via T tube. A postoperative T tube cholangiogram after cholecystectomy in a 62 year old patient showed two retained calculi of 14 mm diameter each. Endoscopic sphincterotomy was not possible because of previous Billroth II gastrectomy. The stones were disintegrated by electromagnetically generated extracorporeal shock waves. The T tube was replaced by a balloon catheter and the papilla of Vater was dilatated allowing passage of stone fragments. We conclude that retained bile duct stones can be removed without sphincterotomy or percutaneous extraction by extracorporeal shock wave lithotripsy and papillary dilatation. This method should be considered especially in patients with Billroth II gastrectomy.
Granulocyte colony-stimulating factor in cerebrospinal fluid from patients with meningitis. Granulocyte colony-stimulating factor (G-CSF) in the cerebrospinal fluid from patients with meningitis was measured by our modified enzyme-linked immunosorbent assay for G-CSF. The minimal detection level was 20 pg/mL G-CSF. In patients with bacterial meningitis, the G-CSF levels in the cerebrospinal fluid were extremely elevated, showing a mean value of approximately 1,500 pg/mL. On the other hand, G-CSF levels in the cerebrospinal fluid from 67% patients with aseptic meningitis were moderately increased, showing a mean value of about 80 pg/mL, whereas G-CSF levels in 33% samples remained undetectable. The G-CSF levels and neutrophil counts in the cerebrospinal fluid were proven to be related by Spearman's rank correlation coefficient analysis (r = .724). These elevations of G-CSF levels at inflammation sites associated with bacterial meningitis may indicate that G-CSF plays an important role in the combat of bacterial infections.
Choosing initial therapy for hypertension. A personal view. With numerous safe and effective antihypertensive drugs now available, the clinician should no longer choose only diuretic agents or beta-adrenergic receptor blockers (beta-blockers) as initial therapy. Five classes of agents, including angiotensin converting enzyme inhibitors, beta-blockers, calcium entry blockers, peripheral alpha 1-adrenergic receptor blockers, and thiazide diuretic agents, are all appropriate monotherapy in properly selected patients. The choice depends on efficacy, side effects, demography, comorbidity, dosage schedule, cost, mechanism of drug action, and the pathophysiology of the patient's hypertension. Extensive data are now available that will assist the clinician in choosing an agent that has the greatest probability of success without the need for extensive biochemical or hemodynamic evaluation.
Variations in acromial ossification simulating infant abuse in victims of sudden infant death syndrome. A variation in ossification of the acromial process of the scapula is described. Postmortem radiographs, obtained in 78 infants who died of sudden infant death syndrome, showed an ossific opacity adjacent to the acromial process in 10 infants (13%). This finding was noted bilaterally in six patients and unilaterally in four patients. No two ossicles were identical. Histologically, no growth plate cartilage was evident between the bony structure and the acromion proper; therefore, this appeared to represent a "pseudoepiphysis." Superficially, this normal variation may appear similar to an acromial fracture resulting from infant abuse. However, a careful analysis of the findings of this normal variation should help prevent any confusion with inflicted injury.
Results of treatment and lessons learned from pathologically staged T4 non-small cell lung cancer. Depending on the local extension of primary non-small cell lung cancer (NSCLC) and invaded T4 structure(s), 49 patients underwent complete (CR, n = 14) or palliative (PR, n = 13) resection of exploratory thoracotomy (ET, n = 22) between January 1982 and June 1988. Thoracic radiotherapy (TR) was given to all patients receiving PR (median dose, 43 Gy) and ET (median dose, 53 Gy). With a median follow-up of 44 months, overall 2- and 5-year survival was 25 and 5%, respectively. Patients undergoing ET plus TR had a significantly worse survival than those treated by CR (P = 0.041) and PR plus TR (P = 0.046). Only completely resected patients became long-term survivors (5-year survival, 29%) and significant predictors of their survival were previous weight loss, hemoglobin, and creatinine level, in univariate analysis, and previous weight loss in multivariate analysis. The site of initial treatment failure was mainly local for PR plus TR (85%) and systemic for CR (71%) and ET plus TR (86%). Presented results suggest that surgery might play a role for selected patients with T4 NSCLC, but advances in systemic and local therapy are necessary.
Cardiovascular risk factors in young snuff-users and cigarette smokers. We studied cardiovascular risk factors in 21 young men who were habitual snuff-users, and compared them with the same risk factors in 18 non-tobacco-users and 19 cigarette smokers of the same age and body mass index. Both snuff-users and smokers showed increased levels of alcohol and coffee consumption and a decreased level of physical exercise compared to non-users. Both groups of tobacco-users showed increased serum insulin levels compared to the control group at similar blood glucose concentrations. In contrast to the smokers, snuff-users showed no significant elevation of diastolic blood pressure, haemoglobin concentrations, white cell count, serum cholesterol or triglyceride levels. Snuff users had higher plasma fibrinogen levels than non-users (P = 0.07). The use of snuff by young men appears to have less impact than smoking on cardiovascular risk factors, with the possible exception of elevated serum insulin and plasma fibrinogen levels.
The long QT syndrome. Prospective longitudinal study of 328 families. BACKGROUND. The Long QT Syndrome (LQTS) is an infrequently occurring familial disorder in which affected individuals have electrocardiographic QT interval prolongation and a propensity to ventricular tachyarrhythmic syncope and sudden death. We prospectively investigated the clinical characteristics and the long-term course of 3,343 individuals from 328 families in which one or more members were identified as affected with LQTS (QTc greater than 0.44 sec1/2). METHODS AND RESULTS. The first member of a family to be identified with LQTS, the proband, was usually brought to medical attention because of a syncopal episode during childhood or teenage years. Probands (n = 328) were younger at first contact (age 21 +/- 15 years), more likely to be female (69%), and had a higher frequency of preenrollment syncope or cardiac arrest with resuscitation (80%), congenital deafness (7%), a resting heart rate less than 60 beats/min (31%), QTc greater than or equal to 0.50 sec1/2 (52%), and a history of ventricular tachyarrhythmia (47%) than other affected (n = 688) and unaffected (n = 1,004) family members. Arrhythmogenic syncope often occurred in association with acute physical, emotional, or auditory arousal. The syncopal episodes were frequently misinterpreted as a seizure disorder. By age 12 years, 50% of the probands had experienced at least one syncopal episode or death. The rates of postenrollment syncope (one or more episodes) and probable LQTS-related death (before age 50 years) for probands (n = 235; average follow-up 54 months per patient) were 5.0% per year and 0.9% per year, respectively; these event rates were considerably higher than those observed among affected and unaffected family members. CONCLUSIONS. Among 232 probands and 1,264 family members with prospective follow-up, three factors made significant independent contributions to the risk of subsequent syncope or probable LQTS-related death before age 50 years, whichever occurred first (Cox hazard ratio; 95% confidence limits): 1) QTc (1.052; 1.017, 1.088), 2) history of cardiac event (3.1; 1.3, 7.2), and 3) heart rate (1.017; 1.004, 1.031). The findings from this prospective longitudinal study highlight the clinical features, risk factors, and course of LQTS.
Relationship between foot transcutaneous oxygen tension and ankle systolic blood pressure at rest and following exercise. To determine whether foot transcutaneous oxygen tension (TcPO2) and ankle systolic blood pressure (SBP) measure similar aspects of peripheral vascular occlusive disease (PVOD), the authors examined their relationship at rest and following treadmill exercise. Thirty-seven PVOD patients (mean age 69.2 +/- 0.8 years) rested supine for twenty minutes, followed by a progressive treadmill walking test at a constant speed of 2 mph. The initial grade was 0%; this increased 2% every two minutes until maximal claudication pain (n = 19) or until the occurrence of such limiting symptoms as volitional fatigue (n = 6), ST segment depression (n = 4), dyspnea (n = 3), multiple premature ventricular contractions (n = 2), and angina (n = 2). Patients then rested supine for fifteen minutes. Foot TcPO2 was recorded before, during, and after exercise, whereas ankle SBP was measured before and after exercise. At rest, a curvilinear relationship was found between foot TcPO2 and ankle SBP (foot TcPO2 = 41.89 + 0.22(ankle SBP) + 0.0005 (ankle SBP2); SEE = 9.2, R = 0.64, R2 = 0.41, p less than 0.001). In contrast, the relationship was stronger and more linear during recovery, particularly at the sixth minute (foot TcPO2) = 8.33 + 0.35 (ankle SBP); SEE = 13.6, R = 0.86, R2 = 0.73, p less than 0.001). At rest, foot TcPO2 and ankle SBP characterized different aspects of PVOD because they shared only 41% common variance. During recovery, they provided similar information because up to 73% of the variance was shared. It is concluded that foot TcPO2 should also be used to assess PVOD patients because unique information is obtained at rest and values can be recorded during exercise.
Long-term effectiveness of nonpharmacological treatment of hypertension. Various nonpharmacological therapies reduce the blood pressure of a significant portion of both patients with established hypertension and people who are considered to be at high risk for the development of hypertension; that is, they are secondary and primary preventions. Of the various therapies, weight reduction, sodium restriction, moderation of alcohol intake, and regular aerobic exercise have been shown to be most effective, whereas the effectiveness of other practices has been less uniform or smaller in degree. Changes in lifestyle, mainly involving diet and physical activity, should be widely used to treat and, hopefully, to prevent hypertension.
Nonspecific findings on MR imaging. The importance of correlative studies and clinical information. Although magnetic resonance (MR) imaging is highly sensitive for the detection of abnormalities of the musculoskeletal system, changes in marrow and soft-tissue signal intensity are often nonspecific, and benign conditions may be mistaken for malignancy. In an effort to identify the cause of this type of misdiagnosis in MR examinations, the MR scans were reviewed of 30 patients whose images manifested bone and/or soft-tissue changes that were initially believed to be consistent with malignancy but were later found to represent benign conditions. Of the 30 patients, MR abnormalities were attributable to trauma in 11, benign tumors and tumorlike conditions in ten, infection in seven, and prior radiotherapy in two. In 24 cases, correlative imaging studies (14 patients), appropriate clinical history (six patients), and/or physical and laboratory examinations (four patients) would have avoided these misdiagnoses.
Ureteral catheter tip separation: potential risk using the open-end Flexi-Tip ureteral catheter. Ureteral catheters are a vital part of the urological armamentarium. We report 2 cases in which the flexible tip portion of an open-end Flexi-Tip ureteral catheter became disjointed from the shaft within the renal collecting system during endourological procedures. The etiology, prevention and management of this complication are discussed.
Surgical repair of rectovaginal fistulas in patients with Crohn's disease: transvaginal approach. The surgical management of rectovaginal fistulas complicating Crohn's disease has been associated with unacceptably high failure rates. We sought to modify the available surgical techniques to provide a solution to this challenging problem. Between December 1983 and January 1990, 14 patients with Crohn's disease underwent repair of a rectovaginal fistula. A modified transvaginal approach was employed by the authors. A diverting loop ileostomy was performed on all patients, either as the initial step in the staged management of intractable perianal disease or concurrent with the repair of the rectovaginal fistula. The fistula was completely eradicated in 13 of the 14 women and did not recur during the mean follow-up period of 55.0 months (range, 3-77 months). Intestinal continuity was reestablished in these 13 patients within 6 months after the initial fistula repair. One patient with a very low-lying fistula constituted our only failure. We have found the transvaginal method preferable to the transanal approach because of the relative ease in raising the vaginal flap as compared with a flap of fibrotic and inflamed anorectal mucosa. On the basis of this study, we conclude that a modified transvaginal approach is an effective method for repair of rectovaginal fistulas secondary to Crohn's disease.
Malignant neoplasia of the paranasal sinuses associated with mucocele. Paranasal sinus mucoceles are histologically benign and typically cause ophthalmic morbidity only after significant orbital extension. The authors describe seven patients who had both a mucocele and a malignant tumor in the same paranasal sinus and in whom the neoplasm was discovered incidentally during operation for mucocele. Although malignancy coexistent with paranasal sinus mucocele is uncommon, the association should be considered and appropriate biopsy specimens obtained if operation for mucocele is required.
Detection of micrometastatic tumor cells in bone marrow of breast carcinoma patients. To determine the incidence and clinical significance of micrometastases in the bone marrow of breast carcinoma patients, we performed an immunoalkaline phosphatase assay using anticytokeratin (AE1, AE3, MAK-6) and antiepithelial (113F1, 260F9, 317G5) antibodies on the bone marrow aspirates of 71 stage IV disease patients with either recurrent regional or distant metastases. Although we detected tumor cells within the bone marrow of 38% of these patients with this assay, no significant correlation was seen with patient's age, menopausal status, bone scan, bone marrow core histology, response to induction chemotherapy, number of metastatic sites, dominant site of metastasis, or subsequent clinical outcome. The clinical parameters that were associated with improved survival were one dominant site of metastatic disease and regional soft tissue recurrence without distant disease.