shukoor65/lab-rag-model huggingface dataset:
Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome. We report the fourth case of partial status epilepticus (SE) in benign epilepsy of childhood with rolandic spikes (BECRS). The child suffered long-lasting attacks involving the mouth and pharynx, clinically manifest as speech arrest, sialorrhea, and drooling. Both clinical and electroencephalogram (EEG) data were compatible with the diagnosis of BECRS. Only during SE was the clinical picture similar to that observed in the operculum or Foix-Chavany-Marie syndrome. SE remission was obtained with the usual antiepileptic drug therapy (diazepam, clobazam, valproate). EEG records showed additional patterns of continuous spike-waves during slow sleep and specific inhibition and blocking of interictal centrotemporal spikes by mouth and/or tongue voluntary movements.
Differences in electrophysiological substrate in patients with coronary artery disease and cardiac arrest or ventricular tachycardia. Insights from endocardial mapping and signal-averaged electrocardiography. BACKGROUND. Many studies have combined patients with hemodynamically well-tolerated ventricular tachycardia (VT) and those with cardiac arrest (CA) as a single, homogenous group. Recent studies suggest that these two groups have different electrophysiological substrates and responses to therapy. Most of these studies, however, enrolled patients with a variety of cardiac diagnoses. METHODS AND RESULTS. We used signal-averaged electrocardiography (SAECG) and endocardial catheter mapping to define the electrophysiological substrate in patients with coronary artery disease and VT or CA and correlate the results of the two methods. We also examined the usefulness of SAECG in CA patients to differentiate those with inducible arrhythmias from those who are noninducible. VT patients were more likely to have had a prior myocardial infarction (p = 0.0005) and to have inducible arrhythmias (p = 0.0001) than were CA patients. The induced arrhythmias in patients who presented with VT was VT in more than 90% of cases, whereas in CA patients, polymorphic ventricular tachycardia (PMVT) accounted for one third of induced arrhythmias. Mean filtered QRS duration was longer (135 versus 120 msec) and the terminal QRS voltage was smaller (20 versus 34 microV) in VT than in CA patients (p less than 0.01). Sixty-three percent of CA patients and 87% of VT patients had abnormal SAECG (p = 0.001). VT patients had more extensive endocardial abnormalities and more abnormal (53% versus 40%, p = 0.002), fractionated (8% versus 3%, p = 0.02), late (17% versus 8%, p = 0.0003), and late abnormal or fractionated (14% versus 4%, p = 0.0001) sites than CA patients. VT patients had a greater duration of the longest electrogram (129 versus 109 msec, p = 0.0006) and total endocardial activation time (68 versus 54 msec, p = 0.009). Among CA patients, those with induced VT had more extensive substrate than did those with induced PMVT and were similar to VT patients with induced VT. Among CA patients, the trend for more patients with inducible VT (77%) or PMVT (55%) than noninducible patients (47%) to have an abnormal SAECG did not reach statistical significance (p = 0.14). The positive and negative predictive values of an abnormal SAECG were 77% and 44%, respectively. CONCLUSIONS. VT patients have more extensive endocardial substrate than CA patients, which translates into greater and more frequent SAECG abnormalities. Among CA patients, there are significant differences in substrate between patients with induced VT and those with induced PMVT. SAECG is not useful in differentiating CA patients who have inducible VT or PMVT from those who do not.
Ureteral pseudodiverticula: frequent association with uroepithelial malignancy. Ureteral pseudodiverticula are tiny outpouchings usually seen as incidental findings on retrograde urography during routine work-up for common urinary tract disorders. We report 37 patients with ureteral pseudodiverticula, of whom 17 (46%) had associated uroepithelial malignancy. Ureteral pseudodiverticula were diagnosed in all patients on excretory or retrograde urography as an incidental finding during evaluation for a variety of urologic symptoms and signs. All patients were assessed with urinalysis, urine culture, cystoscopy, and when indicated, ureteroscopy. Diagnosis of transitional cell carcinoma was made by surgery or endoscopic biopsy in 16 patients and squamous cell carcinoma in one. In nine patients with radiographs showing ureteral pseudodiverticula and a urinary tract filling defect (usually in the bladder), 78% of the cases were proved malignant. In six patients with ureteral pseudodiverticula and a stricture in the ureter or renal collecting system, 67% of the cases were proved malignant. Of the patients with tumor, 53% had a single ureteral pseudodiverticulum. In two cases, the pseudodiverticula antedated the diagnosis of malignancy by 2 and 4 years, respectively. Our experience suggests that the association between ureteral pseudodiverticula and uroepithelial malignancy may be more common than previously assumed, approaching 50%. Even a single ureteral pseudodiverticulum should be regarded as a serious marker for potential malignancy, antedating cancer by 2-10 years, and such patients should be closely followed up. The coexistence of ureteral pseudodiverticula and a stricture or filling defect in the urinary tract is highly suggestive that a tumor is present.
Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive. A 4-month-old male infant had a 2-month history of an exfoliative erythroderma and alopecia. Recurrent mucosal infections, diffuse lymphadenopathy, hepatosplenomegaly, lymphocytosis and eosinophilia, anemia, and failure to thrive later developed. Investigation revealed a combined immunodeficiency with T cells of an unusual phenotype in his peripheral blood, skin, and lymph nodes. Our patient's clinical manifestations most closely resemble Omenn's syndrome, a rare form of autosomal recessive combined immunodeficiency.
Methodologic issues in maintenance therapy clinical trials. In the early 1980s, the National Institute of Mental Health supported a multicenter, randomized, controlled, clinical trial on unipolar and bipolar disorder to evaluate the comparative efficacies of lithium carbonate, imipramine hydrochloride, a lithium-imipramine combination, and placebo in preventing the recurrence of affective disorders. The objective of this report is to present a reanalysis of the relative efficacies of these treatments in patients with unipolar disorder to focus attention on general issues related to the design and conduct of maintenance therapy trials. We show that the earlier conclusions of that study that imipramine and the combination therapy are more effective than lithium and placebo in preventing the recurrence of depression in unipolar patients can be accounted for by alternative explanations that are a consequence of the design of the study. Our findings have important implications for the design, conduct, and interpretation of results of maintenance therapy clinical trials in general.
Effects of immunosuppressive therapy in biopsy-proved myocarditis and borderline myocarditis on left ventricular function. Twenty patients with decreased left ventricular (LV) function and endomyocardial biopsy-proved myocarditis (9 patients) or borderline myocarditis (11 patients) were studied to determine whether these 2 histologic subsets of patients with inflammatory heart disease differed in their response to a 6- to 8-week course of immunosuppressive therapy. All patients received a regimen of prednisone, 1.0 mg/kg/day, and azathioprine, 1.5 mg/kg/day, followed by repeat endomyocardial biopsy and reevaluation of LV function. LV function improved significantly in the group with borderline myocarditis, as assessed by LV stroke work--end-diastolic volume ratio (0.26 +/- 0.17 to 0.54 +/- 0.31 kg.m.ml-1, p less than 0.02), heart rate corrected velocity of circumferential shortening (0.49 +/- 0.30 to 0.80 +/- 0.29 circ.s-1, p less than 0.05), and LV ejection fraction (0.30 +/- 0.15 to 0.47 +/- 0.13, p less than 0.05). LV end-diastolic and end-systolic volume indexes also decreased significantly from 129 +/- 40 to 94 +/- 38 (p less than 0.05) and 90 +/- 37 to 49 +/- 26 ml (p less than 0.02), respectively. No significant change in these indexes of LV function or volume occurred in the myocarditis group. Whereas salutory improvements in cardiac output and filling pressures were found in both groups, objective improvement in LV function assessed by complementary indexes of contractility was greatest in the borderline myocarditis group. It is concluded that short-term immunosuppressive therapy improves LV contractile function and appears to be associated with regression of ventricular dilatation in patients with borderline myocarditis to a greater extent than patients with myocarditis.
Monitoring of upper oesophageal sphincter pressure in children. In children technical limitations of upper oesophageal sphincter manometry have restricted investigation to the pull through technique under sedation. In this study we have used an adapted sleeve manometric technique for upper oesophageal sphincter monitoring in unsedated children and determined the influence of the state of arousal on upper oesophageal sphincter pressure. Twenty six children aged 3 to 42 months (median 17.5 months), who were referred for evaluation of oesophageal motor function, were studied with dual sleeve manometric assemblies which monitored upper and lower oesophageal sphincter pressures simultaneously. Pharyngeal, oesophageal body, and gastric pressures were also monitored with seven perfused side holes. Recordings were made for four hours after a meal and were technically successful in 24 children. The child's state of arousal was scored every 12th minute as follows: (A) resting, eyes closed, (B) resting, eyes open, (C) moving but comfortable, (D) restless and uncomfortable, (E) crying. In 67% of the 12 minute samples the children showed good adaptation to the procedure (arousal states A to C). There was a highly significant difference in upper oesophageal sphincter pressure between each of the arousal states (p less than 0.0001), being lowest in category A at (mean (SD) 18.1 (10.3) mmHg and highest in category D 55.7 (13.2) mm Hg. Abrupt changes in the state of arousal were associated with equally abrupt changes in upper oesophageal sphincter pressure. The state of arousal of unsedated children has an important influence on upper oesophageal sphincter pressure. It is essential that this factor is controlled for in any studies of upper oesophageal sphincter tone in children. The sleeve technique is capable of monitoring upper oesophageal sphincter motility for prolonged periods in unsedated children.
Long-term function in University of Cape Town prostheses in the tricuspid position. A 55-year-old woman undergoing triple-valve replacement was found at operation to have a well preserved and functioning University of Cape Town prosthesis that had been inserted 18 years earlier for tricuspid stenosis. Case reporting, valve reappraisal, and factual updating on another case, the world's first documented tricuspid valve replacement for Ebstein's anomaly, are presented. These two remarkable in vivo successes speak well for this historic collar stud valve.
A simple biological index for detection of alcoholic liver disease in drinkers. To make liver biopsy unnecessary in certain cases, PGA (P, prothrombin time; G, gamma-glutamyl transpeptidase; A, apoliprotein AI), a simple biological index combining a specific test for severe liver disease (prothrombin time), a sensitive test of alcoholic liver disease (serum gamma-glutamyl transpeptidase), and a test for liver fibrosis (serum apolipoprotein AI), was evaluated in a training sample of 333 drinkers and validated in 291 other drinkers. All patients underwent an intercostal liver biopsy, and the specimen was independently read by two pathologists. The PGA index varied from 0 to 12. When PGA was less than or equal to 2, the probability of cirrhosis was 0% and the probability of normal liver or minimal changes 83%. Conversely, when PGA was greater than or equal to 9, the probability of normal liver or minimal changes was 0% and the probability of cirrhosis 86%. These values did not vary between training and validation periods, between asymptomatic vs. symptomatic subjects or between PGA at admission vs. PGA 1 week later. This index could be useful for general practitioners in identifying subjects at high risk for severe alcoholic liver disease.
Pain in sickle cell disease. Rates and risk factors. BACKGROUND AND METHODS. Acute episodes of pain are the principal symptom of sickle cell disease, but little is known about the epidemiologic features of these episodes or risk factors for them, nor is it known whether patients with high rates of such episodes die prematurely. We prospectively studied the natural history of sickle cell disease in 3578 patients ranging from newborns to persons up to 66 years old who were followed at clinical centers across the United States. RESULTS. There were 12,290 episodes of pain in 18,356 patient-years. The average rate was 0.8 episode per patient-year in sickle cell anemia, 1.0 episode per patient-year in sickle beta 0-thalassemia, and 0.4 episode per patient-year in hemoglobin SC disease and sickle beta(+)-thalassemia. The rate varied widely within each of these four groups--e.g., 39 percent of patients with sickle cell anemia had no episodes of pain, and 1 percent had more than six episodes per year. The 5.2 percent of patients with 3 to 10 episodes per year had 32.9 percent of all episodes. Among patients with sickle cell anemia who were more than 20 years old, those with high rates of pain episodes tended to die earlier than those with low rates. High rates were associated with a high hematocrit and low fetal hemoglobin levels. alpha-Thalassemia had no effect on pain apart from its association with an increased hematocrit. CONCLUSIONS. The "pain rate" (episodes per year) is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20. Even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival. This outcome is particularly encouraging to investigators studying hydroxyurea and other treatments designed to increase the fetal hemoglobin level.
Peritonitis after cecal perforation. An experimental model to study the therapeutic role of antibiotics associated with allopurinol and catalase. This work uses cecal perforation on the rat as a model of intra-abdominal sepsis. Under these conditions, various antibiotics were tested alone or in association with free radical scavengers, such as allopurinol and catalase. It can be concluded that the scavengers were not effective alone, but when combined with antibiotics they rendered good results in the majority of the groups when given postsepsis. Further studies are needed to determine the real role of agents like the free radical scavengers in infectious situations such as the one discussed here.
Transient corneal opacification induced by cold in Raynaud's disease. A 48-year-old man presented with longstanding complaints of transient blurring of vision on exposure to cold temperatures. A review of family history was noteworthy in that two of the patient's four sons and the patient's brother had similar complaints. All affected individuals had Raynaud's disease. Results of ophthalmic evaluation showed transient corneal opacities. Slit-lamp video photography under cold stress demonstrated conjunctival vascular changes consistent with Raynaud's phenomenon. An extensive work-up for systemic disease was otherwise negative. To the best of the authors' knowledge, anterior segment changes have not been described previously in idiopathic Raynaud's disease.
Hypopharyngeal lipoma as a cause for sudden asphyxial death. Lipomas of the hypopharynx are uncommon tumors that are rarely life threatening. When positioned in the hypopharynx, asphyxia may be the result of the tumor size or sudden displacement into the oropharynx. We present a case of a large hypopharyngeal lipoma, review the literature, and discuss mechanisms by which these lesions may cause sudden asphyxial death.
Systematic underreporting of cutaneous malignant melanoma in Massachusetts. Possible implications for national incidence figures. An independent tabulation of incidence of cutaneous malignant melanoma in Massachusetts indicates that 12% and perhaps as many as 19% of new cases of cutaneous malignant melanoma in Massachusetts are not recorded in the Massachusetts Cancer Registry, significantly more than the expected 5% (p = 0.0001). The increasing number of nonhospital medical settings in which melanomas can be diagnosed and/or treated appears to account for this discrepancy. We suspect that these findings in Massachusetts also apply to cancer reporting systems in other regions of the United States. We suggest that the true incidence of cutaneous malignant melanoma in Massachusetts, and perhaps in the United States, may be significantly higher than reported.
The gastropulmonary route of infection--fact or fiction? Published studies relating to whether medicinal stress-bleeding prophylaxis leading to an increase of gastric pH favors the development of bronchopulmonary infections are reviewed. Results from studies in healthy humans, patients with ulcer disease, and patients in the intensive care unit (ICU) clearly show that the risk of gastric bacterial colonization significantly increases relative to increasing gastric pH. Moreover, a drug-induced increase of gastric pH leads directly to gastric bacterial colonization also in patients in the ICU, above all with bacteria typical of the gastrointestinal tract. Comparing the different bacterial spectra of the oropharynx, stomach, and upper small intestine, it becomes clear that the stomach is a reservoir of bacteria independent of the oropharynx and also subject to retrograde colonization due to the duodenogastric reflux. Both by means of microbiological and in particular direct detection procedures, it can be demonstrated that in at least 30-40% of intubated patients a gastropulmonary route of colonization occurs. In patient groups without a medication-induced increase of gastric pH the number of bacteria detected in the tracheal secretion is about 33% less than in the case of conventional stress-bleeding prophylaxis. These findings make it understandable that a highly significant increase in the pneumonia rate is seen in patients receiving pH-increasing stress-bleeding prophylaxis versus control groups without therapy essentially influencing gastric pH. A risk score was developed that allows an easy description of those patients who are at an increased risk of pulmonary infections due to the gastropulmonary route of colonization.
Improved mammographic accuracy. Use of ancillary imaging studies to evaluate suspicious screening mammograms can improve their positive predictive value for detecting malignant lesions. In 1989 8181 screening mammograms were performed at the breast evaluation center at Memorial Hospital Medical Center in Long Beach. A total of 670 patients were called back for magnification views or ultrasonographic evaluation, and 92 of these patients underwent surgical biopsy. There were 42 cancers diagnosed, yielding an accuracy rate of 46%.
Complications of diverticular disease and non-steroidal anti-inflammatory drugs: a prospective study. Previous retrospective studies have suggested an association between consumption of non-steroidal anti-inflammatory drugs (NSAIDs) and the complications of diverticular disease. Ninety-two patients were entered into a prospective study of the complications of diverticular disease over a 3 year period; 31 were taking NSAIDs, compared with only four age- and sex-matched controls from a representative general practice (P less than 0.001). A second control group comprised 306 patients with cancer of the colon in whom NSAID consumption was again significantly lower than in patients with diverticular disease (22 of 306 versus 31 of 92, P less than 0.001). Of the 31 patients taking NSAIDs, 19 presented with a perforation or peritonitis. By contrast, only eight of the 61 patients not taking NSAIDs had such complications (P less than 0.001). Eleven patients presented with bleeding of whom five were taking NSAIDs and six were not. Patients admitted with complications of diverticular disease have a high incidence of NSAID intake, and it appears that NSAID consumption is associated with a more severe form of the disease.
Heparin, deep venous thrombosis, and trauma patients. One hundred consecutive patients with multiple trauma, who were admitted to a level I trauma center with an injury severity score of 10 or greater, were studied prospectively. A duplex scan was used to evaluate each patient initially and at set intervals during the hospitalization for the presence of deep vein thrombosis (DVT). The prophylactic regimen for DVT in this study was 5,000 U of subcutaneous heparin every 12 hours. This was used in 50 patients at the discretion of the attending physician, while the remaining 50 patients received no DVT prophylaxis. Fourteen of 50 patients (28%) who were receiving heparin developed DVT, while only 1 patient (2%) of the 50 who did not receive heparin developed DVT. The use of heparin did not provide any significant protection in the susceptible trauma patient. It is believed that those patients with minimal lower extremity injuries, lower injury severity scores, and a shorter period of immobilization do not require any form of DVT prophylaxis. However, those patients at increased risk for DVT are better served with either increased doses of heparin or alternative forms of DVT prophylaxis.
Prospective randomized evaluation of ciprofloxacin versus piperacillin plus amikacin for empiric antibiotic therapy of febrile granulocytopenic cancer patients with lymphomas and solid tumors. The European Organization for Research on Treatment of Cancer International Antimicrobial Therapy Cooperative Group. Empiric therapy for febrile granulocytopenic patients is mandatory, but whether monotherapy is a safe alternative and whether fluoroquinolones are useful agents for this indication are still controversial issues. The use of monotherapy with intravenous ciprofloxacin (200 to 300 mg every 12 h) was evaluated against combined therapy with piperacillin plus amikacin in febrile granulocytopenic patients with solid tumor or lymphoma. The study was discontinued prematurely because patients treated with ciprofloxacin had a significantly lower overall success rate than patients treated with piperacillin plus amikacin (31 of 48 patients [65%] versus 48 of 53 patients [91%], P = 0.002). Patients with gram-positive coccal bacteremia had a particularly poor outcome: therapy failed for six of eight patients (75%) treated with ciprofloxacin, while therapy failed for none of four patients treated with piperacillin plus amikacin. Death from primary infection during initially randomized protocol therapy occurred in 7 of 48 patients (14.5%) treated with ciprofloxacin and in 3 of 53 (6%) treated with piperacillin plus amikacin. This study does not support the use of this dose of intravenous ciprofloxacin as empiric monotherapy for fever in granulocytopenic patients.