shukoor65/lab-rag-model huggingface dataset:
Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas? Between 1970 and 1989, 17 children with histologically malignant intracranial ependymomas received treatment at the University of Pennsylvania (Philadelphia, PA). Eleven were treated with prophylactic cranial or craniospinal irradiation plus a local boost (CS-XRT), five with local (L-XRT) irradiation only, and one was treated without (NRT) irradiation. With a median survival of 2 years and a median follow-up time for long-term survivors of 6.0 years, five of 11 patients who received CS-XRT are alive compared with none treated with L-XRT and none treated with NRT. Two-year actuarial survival rates are 40% (L-XRT) and 52% (CS-XRT). When examined for other factors, age and local radiation dose remain the most significant prognostic indicators of survival. The 2-year actuarial survival for children younger than 4 years at diagnosis is 20% compared with 83% for their older counterparts. Likewise, the 2-year survival for patients treated with local radiation doses over 4500 cGy was 55% compared with 0% for patients treated with lesser doses. To date there are a total of 28 recurrences. All have occurred with local components except for six (unknown) who died before the exact site(s) could be determined. There is no significant difference in the failure rates outside the original tumor bed in the three groups. These data suggest that local relapse remains the most significant component of failure. Because intrinsic and extrinsic factors such as age and radiation dose seem to be interrelated and at least as important as the use of craniospinal irradiation, the need for prophylactic treatment for children with anaplastic ependymoma could neither be substantiated nor refuted. The use of local radiation alone, however, should be restricted to carefully designed clinical trials in which meticulous pretreatment evaluation is performed, and vigilant posttreatment evaluation of the spine and brain is mandatory.
Increased free light chains in the urine from patients with multiple sclerosis. We quantitated free kappa (kappa) and lambda light (L) chains in coded urine specimens from subjects with clinically definite multiple sclerosis (MS) (N = 56), other neurologic diseases (OND) (N = 30), and age-matched normal controls (N = 28). Urine from MS patients showed statistically significant increases in free L chains compared with the other groups, although there was overlap between MS patients and OND patients. The ratio of kappa/creatinine was significantly greater in the relapsing-remitting MS group than in patients with clinically stable MS, OND, and normal controls. Elevated free L chains were usually independent of urinary albumin and beta 2-microglobulin levels. Serial studies showed that urinary free kappa/creatinine ratios were elevated during periods of clinical worsening in seven of eight MS patients and subsequently decreased during clinical recovery. The measurement of free L chains in urine obtained at intervals from MS patients may be useful as a marker to monitor disease activity.
The interaction of plasminogen activator inhibitor 1 with plasminogen activators (tissue-type and urokinase-type) and fibrin: localization of interaction sites and physiologic relevance. Plasminogen activator inhibitor 1 (PAI-1), an essential regulatory protein of the fibrinolytic system, harbors interaction sites for plasminogen activators (tissue-type [t-PA] and urokinase-type [u-PA]) and for fibrin. In this study, anti-PAI-1 monoclonal antibodies (MoAbs) were used to identify interaction sites of PAI-1 with these components. The binding sites of 18 different MoAbs were established and are located on five distinct "linear" areas of PAI-1. MoAbs, binding to two distinct areas of PAI-1, are able to prevent the inhibition of t-PA by PAI-1. In addition, two interaction sites for fibrin were identified on PAI-1. The area located between amino acids 110 and 145 of PAI-1 contains a binding site for both components and its significance is discussed in the context of the t-PA inhibition by fibrin-bound PAI-1. Subsequently, the MoAbs were used to assess the role of platelet-PAI-1 in clot-lysis. An in vitro clot-lysis system was used to demonstrate that clot-lysis resistance is dependent on the presence of activated platelets and that PAI-1 is a major determinant for lysis-resistance. We propose that, upon activation of platelets, PAI-1 is fixed within the clot by binding to fibrin and retains its full capacity to inhibit t-PA and u-PA.
Granulomatous pulmonary zygomycosis in a patient without underlying illness. Computed tomographic appearances and treatment by pneumonectomy. Pulmonary zygomycosis rarely occurs in the absence of underlying disease. We report a patient with granulomatous pulmonary zygomycosis without underlying disease who presented with a pulmonary mass. We present the computed tomographic findings that we believe have not been described previously. We also report the successful treatment by pneumonectomy.
Mucosal dysplasia and DNA content in ulcerative colitis patients with ileorectal anastomosis. Follow-up study in a defined patient group. In a follow-up study of an epidemiologically defined patient group comprising 1,274 patients with ulcerative colitis diagnosed in Stockholm County during 1955-1979, 55 patients had undergone colectomy with ileorectal anastomosis (IRA). Nine of these were found to have Crohn's disease after histopathologic review of the colectomy specimens. Of the 46 patients with ulcerative colitis remaining for evaluation, two died postoperatively. Twenty-five patients were subsequently reoperated with rectal excision owing to intractable inflammatory activity (n = 22, one postoperative death) or owing to dysplasia (n = 3). Of 19 patients with their IRA still intact at time of follow-up, 15 patients (median disease duration 23 years) had a flexible sigmoidoscopy with multiple biopsies performed. The average length of the remaining rectum and sigmoid colon was 26 cm. No patient had findings of dysplasia, carcinoma, or DNA aneuploidy. None of the four remaining patients had developed dysplasia or carcinoma at the time of the latest regular rigid sigmoidoscopy. The risk of malignant transformation in this selected group of patients with ulcerative colitis operated upon with colectomy and IRA derived from an epidemiologically defined population seems to be low.
Is erythrokeratoderma one disorder? A clinical and ultrastructural study of two siblings. Two sisters with erythrokeratoderma are described. In the younger sister the clinical appearance corresponded to erythrokeratoderma variabilis (EKV), whereas in the older sister it corresponded to progressive symmetrical erythrokeratoderma (PSEK). Ultrastructural findings in both cases were identical. We suggest that EKV and PSEK are different manifestations of a single condition.
Oral contraceptives increase insulin-like growth factor binding protein-1 concentration in women with polycystic ovarian disease. Insulin-like growth factor-I (IGF-I) stimulates ovarian androgen production. Insulin-like growth factor binding protein-1 (IGFBP-1) inhibits IGF actions in vitro. OBJECTIVE: To investigate the effect of oral contraceptive (OC) pills, given for 3 months, on serum gonadotropin, androgen, IGF-I, and IGFBP-1 concentrations, and glucose tolerance in seven women with polycystic ovarian disease (PCOD) and in five healthy control subjects. PATIENTS: Seven women with PCOD and five healthy control subjects. INTERVENTIONS: An oral glucose tolerance test (OGTT) was performed before and after treatment with OC. RESULTS: After treatment with OC, serum luteinizing hormone, androstenedione, and free testosterone levels decreased, and sex hormone-binding globulin concentration increased in the women with PCOD as well as in the control subjects. The cumulative response of serum insulin to OGTT was larger in the women with PCOD than in the control subjects both before and after treatment. Serum IGF-I concentration, which was unchanged during OGTT, decreased from basal level of 326 +/- 70 micrograms/L to 199 +/- 28 micrograms/L after treatment with OC in the women with PCOD, whereas no change was found in the control subjects (from 235 +/- 11 micrograms/L to 226 +/- 11 micrograms/L). Treatment with OC caused an increase of the mean basal IGFBP-1 concentration from 24 +/- 7 micrograms/L to 73 +/- 14 micrograms/L in the women with PCOD. This increase was constant during the OGTT. In the control subjects, treatment with OC did not result in any significant change in IGFBP-1 concentrations (from 44 +/- 11 micrograms/L to 61 +/- 9 micrograms/L). CONCLUSION: The combination of decreased total IGF-I concentration and increased IGFBP-1 concentration induced by OC may decrease ovarian androgen production in PCOD.
Immunohistochemical demonstration of CD23 expression on lymphocytes in rheumatoid synovitis. The leucocyte antigen CD23 is expressed by B lymphocytes following activation by a number of stimuli and functions as an IgE receptor, and in its soluble form, as a putative B cell growth factor. The expression of CD23 on the surface of lymphocytes in paraffin wax sections of synovial biopsy specimens was studied using a novel mouse monoclonal antibody, BU38. Specimens were investigated from nine cases of rheumatoid arthritis, six cases of osteoarthritis, and eight cases of chronic inflammation in articular and non-articular tissues. CD23 was expressed on a high proportion of lymphocytes in all forms of chronic inflammation and was not specific for rheumatoid arthritis. It may be a characteristic feature of any chronic inflammatory response. As CD23 was found on the surface of lymphocytes in many cases of these arthritides, sCD23 in serum or synovial fluid may yet prove a useful marker for the severity of the inflammatory infiltrate.
Ulcerative colitis in the autotransplanted neovagina. A 33-year-old woman had undergone a radical surgical excision for carcinoma of the vagina at age 11. Vaginal reconstruction using a pedicled transplant of the sigmoid colon was successfully accomplished at age 19. Fourteen years later she developed vaginal bleeding, and a biopsy of the neovagina revealed changes consistent with ulcerative colitis. Within 2 weeks the patient developed bleeding per ano, and a rectal biopsy also revealed changes consistent with ulcerative colitis. Symptoms responded to sulfasalazine. This is believed to be the first case report of ulcerative colitis developing in colonic tissue used for a neovagina. The etiology, natural history, and surveillance of ulcerative colitis in relation to this unique case are reviewed. This case argues against the hypothesis that chronic ulcerative colitis is causally related to direct exposure to alimentary antigens.
An atrial natriuretic factor analogue at low doses attenuates forearm reflex vasoconstriction to cardiopulmonary receptor deactivation in patients with hypertension. Contrasting data exist about a possible modulation of the autonomic function by atrial natriuretic factor (ANF) in human beings, particularly at low, biologically, significant concentrations. We have evaluated that possibility by increasing plasma ANF levels through the infusion of a synthetic analogue (WY-47,663, anaritide) in five male patients with mild to moderate uncomplicated hypertension. Nonhypotensive lower body negative pressure (-10 mm Hg x 5 min) was used to selectively deactivate cardiopulmonary receptors and to stimulate sympathetic efferent tone reflexogenically. ANF was given at either a low rate (0.005 micrograms/kg/min x 60 min, which was previously shown to increase plasma ANF in a range compatible with physiologic stimuli) or at a high rate (0.05 micrograms/kg/min x 60 min, each). Administration of ANF was preceded and followed by vehicle infusion (Haemacell x 30 min). Forearm blood flow (venous plethysmography), intraarterial blood pressure, and heart rate were monitored continuously, and venous immunoreactive ANF, plasma renin activity, aldosterone level, and venous hematocrit were measured at the end of both control and infusion periods. Arterial norepinephrine values, an indirect index of sympathetic discharge, were measured at rest and during lower body negative pressure conditions. Graded systemic ANF infusion increased immunoreactive ANF and venous hematocrit, decreased aldosterone level and plasma renin activity, whereas resting norepinephrine levels, blood pressure, and heart rate did not change. Lower body negative pressure decreased forearm blood flow during vehicle infusion, but it lost its vasoconstrictor effect during infusion of ANF. To identify the site of that inhibitory action, ANF was also infused into the brachial artery at rates that raised local but not systemic levels of immunoreactive ANF.
Immunological evidence for the co-existence of the Lambert-Eaton myasthenic syndrome and myasthenia gravis in two patients. Two patients are described in whom a clinical and electromyographic diagnosis of the Lambert-Eaton myasthenic syndrome (LEMS) was made. Serum antibodies to voltage-gated calcium channels (VGCCs), the antigenic target in LEMS and to acetylcholine receptors (AChRs), the antigen in myasthenia gravis, were detected at raised titres in both cases, using radioimmunoassays based on 125I-omega-Conotoxin labelled VGCCs and 125I-alpha-Bungarotoxin labelled AChRs. These data provide immunological evidence for the coexistence of the two disorders in these patients.
Fine-needle aspiration biopsy: application to pediatric head and neck masses. Fine-needle aspiration biopsy cytology is widely used to screen masses in adults. The authors present a series of 89 fine-needle aspiration biopsies performed between January 1973 and December 1988 on 86 pediatric patients with clinically significant head and neck masses. All fine-needle aspiration diagnoses were confirmed by subsequent surgical biopsy, autopsy, or clinical follow-up for a minimum of 18 months. Of 21 tumors identified, 19 were malignant. Of the 89 aspirations performed, 67 required no subsequent surgical biopsy. In 11 of these cases, metastatic or recurrent tumor was diagnosed and appropriate therapy instituted. Fifteen of the 89 aspirates revealed previously undiagnosed tumors requiring surgical intervention. One false-negative and two false-positive results were obtained. No radical treatment resulted from the false-positive diagnoses, and no patient delay in treatment occurred because of the false-negative result. The sensitivity was 94.4%, and the specificity was 97.1%. The usefulness and cost-effectiveness of fine-needle aspiration is stressed.
Orthotopic cardiac transplantation in two patients with previous malignancy. This report presents 2 patients with previous malignancy in whom congestive cardiomyopathy developed secondary to doxorubicin toxicity. Both patients underwent orthotopic cardiac transplantation 3 and 5 years ago and are now in functional class I with no evidence of malignant recurrence.
Priapism as a manifestation of isolated genital vasculitis. Vasculitis may affect virtually any organ system in the body. We describe a patient who presented with priapism due to isolated genital vasculitis. This responded promptly to oral corticosteroids. In patients presenting with priapism, a possible underlying vasculitis should be considered in the differential diagnosis; the treatment for this may be nonsurgical.
Acute and chronic symptoms of mononucleosis. BACKGROUND. The clinical symptoms and durations of illness of patients with infectious mononucleosis (IM) are variable and are poorly documented in the scientific literature. METHODS. Patients who presented for care at the Student Health Service of a Canadian university between September 1985 and May 1988 and had been diagnosed as having IM were surveyed. Health experience during the acute and convalescent phases of IM was compared with that of a group of patients matched for age, sex, date of diagnosis, and year of study, who had suffered acute upper respiratory tract infections (URI), other than Epstein-Barr virus (EBV)-induced, during the same period. RESULTS. Students were sicker for longer after IM than after non-EBV-induced URI. During the acute phase of illness, the symptoms of fatigue (P = less than .000001), night sweats (P = .000001), and painful neck swelling (P = .00003) were seen significantly more often in the IM group. The severity and duration of these symptoms were also significantly worse in IM patients. Getting tired easily (P = .002), diurnal somnolence (P = .002), and depression (P = .002) were significantly more common postacute symptoms. Eleven percent of IM patients reported persistence of symptoms longer than 100 days, and in 6% of patients the symptoms had persisted after 1 year. Convalescent cases showed a trend toward reduced alcohol intake and, perhaps, reduced alcohol tolerance. CONCLUSIONS. IM involves excessive morbidity in a student community compared with URI that was other than EBV-induced, during both the acute and the postacute phases of infection.
Dynamic exercise-induced elevation in plasma levels of atrial natriuretic peptide in patients with effort angina pectoris. We investigated the relationship between plasma atrial natriuretic polypeptide (ANP) levels and hemodynamic indices during dynamic exercise testing in 15 patients with effort angina pectoris. Patients exercised on an angina-limited, supine, multistage bicycle ergometer, and plasma ANP levels and hemodynamic indices were measured at rest, at peak exercise, and 6 minutes after exercise. Plasma ANP levels increased significantly at peak exercise. Pulmonary artery wedge pressure (PAWP) and coronary sinus blood flow (CSBF) were significantly correlated with plasma ANP levels before and at peak exercise (PAWP: r = 0.69, p less than 0.001; CSBF; r = 0.45, p less than 0.05). In six of eight patients whose PAWP exceeded 20 mm Hg at peak exercise, plasma ANP levels were increased at 6 minutes after exercise, whereas PAWP had decreased relative to the values obtained at peak exercise. Plasma ANP concentrations at 6 minutes after exercise were not correlated with PAWP at the same time. However, PAWP at peak exercise was correlated with the plasma ANP levels at 6 minutes after exercise (r = 0.80, p less than 0.001). These results suggest that in patients with effort angina pectoris left ventricular dysfunction resulting from exercise-induced myocardial ischemia may increase preload excessively and may contribute to the excess secretion of ANP after dynamic exercise.
Acute appendicitis in patients with AIDS/HIV infection. Nine patients known to have acquired immunodeficiency syndrome (AIDS) and/or human immunodeficiency virus (HIV) infection and operated on for acute appendicitis are presented. Six of the nine patients did not have an elevation in the white blood cell count preoperatively. Two patients underwent diagnostic laparoscopy prior to exploration. In four cases, a perforated appendix was removed. Seven patients had persistent postoperative fever, while all nine had a significant lack of leukocytosis after surgery. Other than the absence of a preoperative increase in white blood cell count, the presenting characteristics of this group were similar to those expected in immunocompetent patients. However, concern for a possible opportunistic infection etiology and a desire to avoid operating on these patients resulted in an undue delay prior to exploration. The use of diagnostic laparoscopy aided in earlier and more accurate diagnosis. Despite persistent postoperative fever, appendectomy was performed in patients with AIDS/HIV infection without significant increase in morbidity and mortality.
Changes in hypertension treatment and in congestive heart failure mortality in the United States. The use of antihypertensive agents has increased in recent years and has been more marked among individuals in older age groups (65-74 years) than in middle-aged groups (45-54 years). Because hypertension is a strong risk factor for the development of congestive heart failure, more common use of antihypertensive agents would be expected to reduce the incidence of congestive heart failure. Examination of the national death statistics reveals decreases in congestive heart failure mortality at younger ages with mortality increases at older ages. The reasons for these divergent trends in congestive heart failure mortality in different ages and the apparent inconsistency with respect to the changes in the use of antihypertensive drugs are explored. We speculate that antihypertensive treatment does not completely prevent congestive heart failure but merely postpones its development by several decades.
Causes of death in patients with tuberous sclerosis. Of the 355 patients with tuberous sclerosis complex (TSC) examined at the Mayo Clinic, 49 had died (9 of causes other than TSC). We attempted to determine what pattern of organ involvement occurred most often in the 40 patients who died of TSC. One baby died of cardiac failure due to cardiac rhabdomyomas, and one child died of rupture of an aneurysm of the thoracic aorta. Eleven patients died of renal disease, which was the commonest cause of death. Ten patients died as a result of brain tumors, and four patients (who were 40 years of age or older) died of lymphangiomyomatosis of the lung. Thirteen patients with severe mental handicaps died of either status epilepticus or bronchopneumonia; in all but one of these patients, the only source of information was the death certificate. Survival curves show a decreased survival for patients with TSC in comparison with that for the general population. Patients with TSC need lifelong follow-up for early detection of potentially life-threatening complications.
Sigmoid sinus thrombosis diagnosis by contrasted MRI scanning. Septic thrombosis of the transverse-sigmoid sinuses and the jugular bulb is a highly lethal condition. The presenting signs and symptoms of this disease entity are subtle and not in proportion to the magnitude of the problem. Later in the disease course, sudden fulminant findings appear. A high index of suspicion, combined with scanning techniques of either enhanced MRI or CT, allows prompt diagnosis and treatment. MRI enhanced with gadolinium-DTPA (Gd) is a valuable adjunct that confirms the diagnosis and delineates the extent of suspected pathology.
Coarctation and hypoplasia of the aortic arch: will the arch grow? Hypoplasia of the transverse aortic arch of various degrees of severity is commonly seen in infants who have coarctation of the aorta. It is more often present when the coarctation is associated with intracardiac lesions that diminish or limit forward flow in the ascending aorta and promote right to left flow through an arterial duct. The increased frequency of surgical treatment of infants with complex coarctation, which is in part related to the ability to stabilize their condition with prostaglandin E1, has posed the question of the potential for growth and development of the originally hypoplastic aortic arch after conventional repair of aortic coarctation. Review of our experience with transverse aortic arch hypoplasia, found in 33 (32%) of 102 infants undergoing coarctation repair by subclavian flap aortoplasty or classic resection and end-to-end anastomosis, revealed excellent growth of the transverse arch after repair in all patients available for linear follow-up. The currently proposed extended arch repair should be reserved for the small group of infants with transverse aortic arch to ascending aorta diameter ratios (arch indices) of less than 0.25.