shukoor65/lab-rag-model huggingface dataset:
Ebstein's anomaly appearing in the neonate. A new surgical approach. Ebstein's anomaly appearing during the neonatal period carries a high mortality rate. These infants exhibit cyanosis, acidosis, and congestive heart failure. The pathophysiologic characteristics consist of severe tricuspid regurgitation and functional pulmonary atresia. As a result of the inability of the right ventricle to generate forward flow through the pulmonary arteries, these infants remain dependent on ductal patency. Since May 1988, five newborn infants with severe Ebstein's anomaly have been admitted for treatment at our institution. At initial examination, they weighed 3.6 +/- 1.8 kg and had a mean oxygen tension of 29.6 +/- 2.3 mm Hg and a mean pH of 7.20 +/- 0.05. Chest roentgenography demonstrated a mean cardiothoracic ratio of 0.81 +/- 0.02. As determined by echocardiography, the right atria were massively enlarged, severe tricuspid regurgitation was present in all patients, and the pulmonary valves were not opening. All infants were dependent on prostaglandin E1 and attempts to wean them from this drug were unsuccessful. Palliative treatment consisted of tricuspid closure with autologous pericardium and an aortopulmonary shunt of 4 mm polytetrafluoroethylene tubing. There were no operative or late deaths. At discharge, mean oxygen tension was 42.2 +/- 0.85 mm Hg and mean systemic oxygen saturation was 83.2% +/- 1.94%. Infants have grown satisfactorily during the follow-up period. Three infants have since returned for further surgical intervention. One infant, at 11 months of age, underwent a Glenn anastomosis for progressive oxygen desaturation. Two infants have returned, at ages 23 and 22 months, for Fontan procedures, which represent their definitive operative management. We believe this new procedure offers excellent palliative treatment for Ebstein's anomaly in critically ill neonates. Feasibility of later definitive correction is demonstrated by the good results obtained with the Fontan procedure in two infants.
Foscarnet in the treatment of cytomegalovirus infection of the esophagus and colon in patients with the acquired immune deficiency syndrome. Foscarnet, administered via a central line, was used for the treatment of 18 episodes of cytomegalovirus (CMV) esophageal ulceration in 15 patients, and in the treatment of 27 episodes of CMV colitis in 22 patients. In 15 of the 18 episodes of the esophageal disease, there was complete loss of symptoms within 2 wk. Only three of the 15 patients responding to foscarnet therapy had a relapse of their esophageal disease. Two of these patients were successfully retreated with foscarnet. Of the 22 patients receiving foscarnet for CMV colitis, four died during foscarnet therapy. Of the 18 patients completing the course of treatment with foscarnet for first-episode CMV colitis, 11 remitted completely and six had a partial remission. All patients with partial remission had a second pathogen responsible for the continuation of their diarrhea. Only one patient completing a course of foscarnet failed to respond microscopically to treatment. Three patients experienced relapse of their CMV colitis, two of whom responded to further courses of foscarnet. Foscarnet is an effective therapy for CMV disease of the gastrointestinal tract, with rapid resolution of clinical symptoms, long-term remission of esophageal disease, and shorter disease-free periods for CMV colitis.
Relationship between glucose tolerance and quantitative changes of islet cells in chronic obstructive pancreatitis due to pancreatoduodenal cancer. This study was conducted to clarify the relationship between indicators of preoperative glucose tolerance and quantitative changes of endocrine cells in the islet of Langerhans in patients with chronic obstructive pancreatitis due to pancreatoduodenal cancer. Twenty-one specimens of pancreatic tissue obtained from patients who underwent pancreatoduodenectomy or total pancreatectomy for pancreatoduodenal cancer were divided into four groups according to the degree of fibrosis (Grades 0-III). Islet cells in serial sections were stained immunohistochemically to determine the proportions of B-, A-, D-, and PP-cells in the islets of Langerhans. In patients with pancreatic tissue with the most severe fibrosis, of several preoperative glucose metabolism indices measured, it was found that the ratio of integrated value of change in serum insulin to that of serum glucose (sigma delta IRI:sigma delta BS) was significantly decreased. There was a significant positive correlation between proportion of B-cells, and both the ratio of change in insulin to that of glucose (delta IRI:delta BS) and sigma delta IRI:sigma delta BS. On the other hand, there was a significant negative correlation between proportion of A-cells and sigma delta IRI:sigma delta BS. The present data strongly suggest that it is possible to estimate the degree of fibrosis and to quantify changes of the islet cells prior to surgery in patients with obstructive pancreatitis due to pancreatoduodenal cancer by calculating these glucose metabolism indices, and that sigma delta IRI:sigma delta BS is a particularly useful index.
Enucleation after plaque radiotherapy for posterior uveal melanoma. Histopathologic findings. A review of 1019 patients with posterior uveal melanomas, who were treated with episcleral plaque radiotherapy between April 1976 and December 1987, showed that 59 (6%) of these patients have thus far required enucleation of the affected eye. The most common clinical reasons for enucleation were tumor regrowth (51%) and neovascular glaucoma (31%). These irradiated melanomas were of mixed cell type in 40 cases (68%), spindle in 10 cases (17%), totally necrotic in 7 cases (12%), and epithelioid in 2 cases (3%). Some degree of tumor necrosis was seen in all cases. Overall, the median number of mitoses per 40 high-power fields in these irradiated tumors was 0. Most (64%) of the tumors had no identifiable mitosis in 40 high-power fields. A three-way simultaneous comparison of the following groups was made: irradiated eyes enucleated for tumor regrowth, irradiated eyes enucleated for neovascular glaucoma, and a matched group of nonirradiated posterior uveal melanomas. This comparison showed that the largest tumor dimension was significantly greater in the irradiated eyes enucleated for tumor regrowth than in the other two groups. Mitotic activity was significantly higher in the irradiated tumor regrowth and nonirradiated groups than in the irradiated neovascular group. There was no significant difference among the three groups in an analysis of height. The results suggested that mitotic activity may exist after plaque radiotherapy, especially when there is clinical evidence of tumor regrowth.
Sepsis. What it is and how to recognize it. Sepsis, shock, and resuscitation may result in various degrees of ischemia-perfusion injury that may produce widespread organ dysfunction through complex interactions and activation of host immunoinflammatory processes. As the pathophysiologic mechanisms of the inflammatory response are better defined, we may be able to modulate the generalized inflammatory state we know as sepsis and prevent the development of multiple organ failure syndrome. At present, however, the mainstay of therapy remains prompt resuscitation to eliminate regions of hypoperfusion and to limit as much as possible those factors that predispose to further organ injury while the source of inflammatory stimulation is being identified and controlled.
Thyroid adenocarcinomas secondary to tissue-specific expression of simian virus-40 large T-antigen in transgenic mice. A hybrid gene comprising the bovine thyroglobulin gene promoter and the coding region for the simian virus-40 large T- and small t-antigens was used to generate 30 transgenic mice by microinjection into the pronuclei of single cell embryos. All animals except three developed, as single primitive pathology, a dramatic enlargement of the thyroid gland. Compression of trachea and esophagus, accompanied by dyspnea, inspiratory stridor, and dysphagia, led to a progressive cachexia and premature death attributed to respiratory failure. Despite the large thyroid volume, T4 levels were abnormally low, and the progression of the syndrome could be delayed by a substitutive treatment with thyroid hormones. The rapid evolution of the disease, leading to the death of most founder transgenic animals before the breeding age, prevented transmission of the transgene to their offspring. Only two transgenic lines are presently surviving. Immunohistochemical analysis of the tissues revealed a specific expression of the simian virus-40 antigens in the thyroid cells. Hyperplasia was already obvious at birth. Older animals displayed moderately to poorly differentiated thyroid adenocarcinomas. Electron microscopy revealed, however, the persistence of cell polarity and the presence of microfollicles between the densely packed cells. Cell lines derived from these large T-expressing thyroids were shown to have lost expression of both thyroglobulin and thyroperoxidase, while expressing low levels of TSH receptors. These transgenic mice could constitute an interesting model of aggressive adenocarcinoma, sharing phenotypical similarities with the anaplastic type of human thyroid tumors.
A phase I trial of high-dose diaziquone and autologous bone marrow transplantation: an Illinois Cancer Council study. Diaziquone (AZQ), a synthetic quinone with demonstrated activity against acute nonlymphocytic leukemia (ANLL), primary CNS tumors, and non-Hodgkin's lymphoma (NHL), is virtually devoid of nonhematopoietic toxicity at conventional doses. As a prelude to its inclusion into bone marrow transplant (BMT) preparative regimens, a phase I study of high-dose AZQ with autologous BMT (ABMT) was performed. Patients with refractory solid tumors and lymphomas were treated with a single 24-hour infusion of AZQ at 50 to 355 mg/m2 in dose escalations of 20%. Fifty-six patients received 69 courses. Those receiving greater than 60 mg/m2 had nadir granulocyte and platelet counts less than 500/microL and 20,000/microL, respectively. Nausea, vomiting, stomatitis, and diarrhea were mild, transient, and not dose-related. Transient minimal elevations of liver function tests were seen in five patients and were also not dose-related. The maximally tolerated dose (MTD) of high-dose AZQ was found to be 245 mg/m2, with nephrotoxicity being dose-limiting. Significant azotemia was seen in four of 12 patients treated at 295 and 355 mg/m2, including fatal anuric renal failure in three of these patients. Reversible proteinuria also occurred in 24 of 26 courses above 150 mg/m2, including nephrotic range proteinuria in eight courses, all at doses of 205 to 355 mg/m2. The proteinuria was also associated with multiple proximal tubular defects including generalized aminoaciduria and proximal renal tubular acidosis. There were six early deaths including two of early renal failure (295 and 355 mg/m2), two of sepsis (205 and 245 mg/m2), one of a pulmonary embolus (85 mg/m2), and one of progressive disease (60 mg/m2). Of 50 patients who were assessable for response, there were seven responses including two of 10 with primary CNS tumors, one of 12 with malignant melanoma, one of five with non-small-cell lung carcinoma, two of two with breast carcinoma, and one of one with ovarian carcinoma. Because of its activity in ANLL and NHL and its unique toxicity spectrum, high-dose AZQ may improve the efficacy of current BMT preparative regimens without significantly increasing their nonhematopoietic toxicity.
Use of purified streptokinase in empyema and hemothorax. Enzymatic debridement of the pleural cavity for retained hemothorax or empyema is a frequently overlooked option. Thirteen of fourteen patients (93%) with retained pleural collections underwent successful enzymatic debridement and tube drainage with purified streptokinase injections. The average increase in chest tube output following streptokinase injections was 158%. No significant adverse reactions occurred. One patient required thoracotomy when streptokinase therapy failed. Two others had successful resolution of their pleural collections but required thoracotomy for other indications. There were two deaths (14%), which were unrelated to the use of streptokinase or residual empyema. Intrapleural streptokinase is a safe, effective means of removing retained proteinaceous collections in the pleural space. It is a useful adjunct to chest tube drainage and may obviate the need for more invasive procedures.
Sexual function and testosterone levels in men with nonalcoholic liver disease. The effects of nonalcoholic liver disease on sexual desire, arousal, activity, orgasmic function and satisfaction and serum testosterone levels were studied in 75 men with nonalcoholic liver disease. Each man was interviewed about his sexual behavior and problems and was asked to comment on whether he felt liver disease affected his sexual function. The average age of the patients was 49 yr, and a wide variety of liver diseases was represented. Child-Pugh grading was A in 51 patients, B in 18 and C in 6; the mean duration of liver disease was 8 yr. Sexual desire, arousal and activity of patients with grade A disease were within the ranges observed in studies of healthy men of comparable age. Diminished sexual desire was reported by 2% of grade A patients and 35% of grade B and C patients (p less than 0.005). Arousal problems were noted by 16% of grade A patients, 60% of grade B patients and 67% of C patients (p less than 0.005). Loss of erection and inability to regain erection were noted by 7%, 40% and 67% of grade A, B and C patients, respectively (p less than 0.01). Premature and retarded ejaculation were more frequent in patients classed in Child-Pugh grades B and C. Frequency of coitus and orgasm were significantly higher in grade A patients than in grade B and C patients. Total and free testosterone levels were (in nanograms per milliliter) A, 677/1.78; B, 416/1.06; and C, 178/0.43 (p less than 0.002). We concluded that Child-Pugh grade A nonalcoholic liver disease in men does not affect sexual desire, function or performance. Men with disease grades B and C have significant sexual dysfunction and significant reduction of both total and free testosterone levels.
Lymphography--current role in oncology. A review of the use of lymphography at this hospital, a major oncology centre, is presented. The advent of computed tomography has brought a dramatic reduction in the number of lymphograms currently performed for diseases such as lymphoma, testicular tumours and gynaecological malignancies. This study analyses the reasons for this decline, and concludes that valuable information can still be obtained from lymphography in certain selected groups of patients.
Comparison of continuous epidural bupivacaine infusion plus either continuous epidural infusion or patient-controlled epidural injection of fentanyl for postoperative analgesia. We compared the postoperative epidural analgesia provided by the continuous epidural infusion of bupivacaine supplemented with patient-controlled injection (PCA) of epidural fentanyl with that provided by a continuous infusion of bupivacaine supplemented with a continuous epidural infusion of fentanyl. Our patient population comprised 16 ASA physical status I or II patients undergoing laparotomy with a midline incision under general anesthesia combined with bupivacaine epidural analgesia. Post-operatively, a continuous epidural infusion of bupivacaine (0.1 mg.kg-1.h-1) was combined with epidural fentanyl given by either (a) PCA (15-micrograms bolus with a lockout interval of 12 min, n = 8) or (b) continuous infusion (1 microgram.kg-1.h-1, n = 8). In the case of inadequate pain relief in the latter group, the fentanyl infusion rate was increased by 10 micrograms/h. Analgesia evaluated by a visual analogue pain score and by a verbal pain score was similarly effective in both groups. The sedation score was also similar in both groups. The total dose of epidural fentanyl administered during the first 24 h was significantly lower in the PCA group than in the continuous infusion group (405 +/- 110 micrograms vs 1600 +/- 245 micrograms, P less than 0.001). The dose of fentanyl given during each 4-h interval ranged between 40 and 160 micrograms in the PCA group and 251 and 292 micrograms in the continuous infusion group. Clinically detectable respiratory depression was not observed in either group. In conclusion, epidural administration of 0.1 mg.kg-1.h-1 bupivacaine combined with fentanyl provides effective postoperative analgesia with a total dose of fentanyl required that is lower when fentanyl is administered by epidural PCA rather than by continuous epidural infusion.
Results of fundoplication in a UK paediatric centre. Of 55 children (age 3 months to 16 years) who had fundoplication, major complications occurred in nine (16 per cent): paraoesophageal hernia (five cases), prolonged ileus (two cases), recurrent gastro-oesophageal reflux (one case), and accidental perforation (one case). The single most important factor resulting in complications was the omission of crural repair; of seven patients without crural repair, five developed paraoesophageal hernia/recurrence. Four patients required repeat fundoplication for severe recurrent symptoms and one of these developed the unusual complication of pericardiogastric fistula. Thirteen patients had strictures before operation from reflux oesophagitis, six (46 per cent) resolved after fundoplication alone, six responded to dilatation (mean five sessions), and one required colon interposition. Our preliminary experience with balloon dilatation was encouraging: three of three patients responded after one dilatation only. These results confirmed the efficacy of surgery in controlling reflux: 100 per cent in the short-term and 89 per cent on a 1-6 year follow-up. Major complications might well be reduced by routine crural repair.
Method for auditing turnround time in histopathology related to user requirements. Histopathology audit schemes have concentrated on the accuracy of diagnosis or the standard of technical work. The most appropriate measure of specimen report time for histopathology was assessed. The simplest method is to calculate the mean or median number of days from receipt of specimens to issue of report, but this takes no account of clinical practice. An alternative approach is to compare the laboratory's performance with the clinicians' requirements. Clinicians were invited to complete a questionnaire specifying the reporting times they required subdivided by tissue type, clinical suspicion of malignancy, and bed occupancy of the patient. The questionnaire showed that there were distinct differences in clinicians' requirements. The turnround time for each category of specimen was calculated and expressed in three ways. The traditional method of quoting a median value showed very few differences among the categories. A comparison of the department's performance with the clinicians' requirements, using an arbitrary cut-off point of satisfying at least 80% of the clinicians' most stringent requirements or an achievement index, however, provided useful information for refining laboratory priorities.
Incidence and treatment of rejection episodes in primary orthotopic liver transplantation under FK 506. FK 506 therapy with low doses of steroids was adequate to control rejection in most liver recipients. Rejection episodes were readily reversed with single IV doses of methylprednisone or hydrocortisone. Short courses of OKT3 (3 to 5 days 5-10 mL) controlled severe rejections. The rate of retransplantation directly due to rejection was low (1.6%). There was a limited need for steroids either early or out to 6 to 12 months.
Concomitant whole-abdominal radiation and intraperitoneal chemotherapy in advanced ovarian carcinoma. A pilot study. Because the use of cisplatin-based chemotherapy for ovarian carcinoma has not significantly improved 5-year survival rates compared with either whole-abdominal radiation (WAR) or single-agent chemotherapy, a pilot study was begun to assess the feasibility of concomitant radiation and chemotherapy. Eleven previously untreated patients with Stages III and IV ovarian carcinoma were treated concomitantly with 2000 cGy of WAR and intraperitoneal (IP) cisplatin followed by additional IP cisplatin after debulking surgery. Toxicity was moderate to severe. Sixty-four percent of patients had Grades 3 to 4 hematologic toxicity, and 36% required hospitalization for sepsis during WAR/IP cisplatin. Hematologic toxicity was less pronounced during IP cisplatin alone. All patients experienced moderate gastrointestinal toxicity. The average percentage of total body weight lost was 13.5%. Fifty-five percent of all patients demonstrated a complete clinical response to therapy, and patients with minimal postoperative residual disease fared better. One patient with persistent disease had acute nonlymphocytic leukemia (ANLL) 24 months after initial diagnosis. No patients with residual disease greater than 20 mm survived, while 50% of patients with less than 20 mm are clinically free of disease. Toxicity appears to be additive with the combination of WAR and IP cisplatin. Therapeutic efficacy was comparable with standard chemotherapy regimens, but no therapeutic or survival advantages were demonstrated with the use of this treatment protocol.
Chagas' heart disease in the United States. BACKGROUND AND METHODS. Chagas' heart disease is believed to be rare in the United States, although many persons from countries where the disease is endemic reside here. We performed a retrospective case review and prospective follow-up of 25 patients with Chagas' heart disease and no obstructive coronary artery disease on angiography. RESULTS. The patients mainly presented with symptomatic atrioventricular block, congestive heart failure, anginal chest pain, sudden death averted by resuscitation, or sustained ventricular tachycardia. Of the 25 patients, 18 had been treated for coronary artery disease or idiopathic dilated cardiomyopathy for up to 108 months before the diagnosis of Chagas' disease was considered. The electrocardiograms frequently suggested coronary artery disease. Six of the seven patients who had exercise thallium-perfusion scans had abnormalities suggesting ischemia or infarction. A left ventricular aneurysm was found in 14 of the 25 patients, segmental akinesia or hypokinesia in 5, and diffuse hypokinesia in 3. Programmed ventricular stimulation performed in 13 patients induced sustained ventricular tachycardia in 9 and nonsustained ventricular tachycardia in 2. Actuarial survival (mean +/- SE) after four years for the entire group was 56 +/- 12 percent; it was 32 +/- 16 percent among those with global left ventricular dysfunction, and 78 +/- 14 percent among those without such dysfunction (P = 0.03). Only patients with left ventricular dysfunction or an aneurysm died (four-year survival, 45 +/- 14 percent, as compared with 100 percent for the remaining patients; P = 0.0002). Heart failure and left ventricular aneurysm or dysfunction were the only independent predictors of death. Nine patients required permanent pacemakers. CONCLUSIONS. In the United States, Chagas' heart disease commonly mimics coronary artery disease or idiopathic dilated cardiomyopathy. The prognosis is poor for patients with heart failure or left ventricular aneurysm or dysfunction. The disease may be underdiagnosed in the United States.