shukoor65/lab-rag-model huggingface dataset:
Lambda-like and V pre-B genes expression: an early B-lineage marker of human leukemias. V pre-B and lambda-like genes are selectively expressed in human pre-B cells and encode polypeptide chains that associate in a mu-pseudolight chain complex that may regulate some crucial steps of early B-cell differentiation. We have followed by polymerase chain reaction and Northern blot analysis the expression of these "pre-B-specific" genes in correlation with the status (rearranged v germline) of Ig gene loci (H, kappa, lambda) in a panel of 32 leukemias pertaining mostly to the B lineage and including a number of ambiguously characterized samples. All cells that had rearranged the H locus only expressed V pre-B and lambda-like transcripts, in agreement with a pre-B status. In this group, some biphenotypic leukemias (mostly My/B) might, in fact, be already engaged in the B lineage. Rearrangement of V kappa or V lambda loci correlated with the disappearance of the pre-B gene products. In a pre-B acute lymphoblastic leukemia cell line that was induced to mature to the B-cell stage in culture upon kappa gene rearrangement, the mu-pseudolight chain complex was actually replaced by the classical mu-kappa molecule. Finally, V pre-B and lambda-like genes were found expressed in two leukemic cells that had retained all Ig loci in germline configuration. This finding raises the possibility of having an early pro-B progenitor in which V pre-B and lambda-like products associate with a H chain surrogate in a complex that would trigger an early event of B-cell differentiation such as the H locus rearrangements.
Erythroderma, palmoplantar keratoderma and profound failure to thrive in an infant. The case is reported of a female infant, who at the age of 3 months developed severe erythroderma, marked hyperkeratosis of the palms and soles and subsequently extreme growth failure and intermittent diarrhoea. Her course was complicated by life-threatening infections but detailed investigation revealed no recognized underlying metabolic or immune abnormality.
Role of interstitial therapy in the treatment of liver cancer. Conventional palliative management of inoperable focal hepatic tumours remains unsatisfactory. Interstitial techniques such as cryotherapy, alcohol injection, low power laser hyperthermia and interstitial radiotherapy offer alternative approaches. Cryotherapy is an effective and precise technique for inducing tumour necrosis. It can only be performed at laparotomy making it relatively invasive and retreatment impractical. Alcohol is cheap and can be injected percutaneously. However, inhomogeneous distribution produces imprecise and nonreproducible lesions. Low power laser hyperthermia produces precise and reproducible areas of necrosis that are roughly spherical in shape. At present, this technique is most effective for small tumours. Interstitial radiotherapy remains the least evaluated of all the interstitial techniques. Unlike cryotherapy and low power laser hyperthermia, the biological effect of ethanol injection and interstitial radiotherapy cannot be monitored in real time by ultrasound. With the exception of cryotherapy, all methods can be applied percutaneously with low morbidity and mortality. None of these techniques is established, but they may offer the prospect of cure in cases where all areas of tumour can be positively identified and fully treated. However, in most instances the intention is to control the growth of relatively small discrete volumes of tumour within the hepatic parenchyma.
Middle ear fluid lysozyme source in experimental pneumococcal otitis media. Middle ear infection with Streptococcus pneumoniae is important in the pathogenesis of acute and chronic otitis media, and lysozyme in middle ear fluid (MEF) is an important inflammatory mediator in this disease. To determine the source of lysozyme during the early period of acute pneumococcal otitis media, chinchillas were irradiated to induce neutropenia, and their middle ears were inoculated with heat-killed, encapsulated pneumococci. The number of inflammatory cells and concentration of lysozyme were measured in MEF between 6 and 72 hours after inoculation. In pneumococcus-inoculated ears, the mean number of inflammatory cells but not lysozyme was significantly lower in MEF from irradiated animals than that from nonirradiated animals at 6 hours. Since lysozyme accumulated in MEF before the influx of inflammatory cells in irradiated animals, the initial release of this inflammatory mediator is most likely not from inflammatory cells; and mucosal epithelial cells, the only other known source of lysozyme in the middle ear environment, were the probable source induced by the direct stimulation of pneumococci. Inflammatory cells may contribute lysozyme later in the inflammatory response, since cellular and lysozyme concentrations in irradiated and nonirradiated animals were similar between 24 and 72 hours. These results suggest that future therapeutic interventions to limit middle ear inflammation in acute otitis media may need to recognize the direct action of pneumococcal cells or their envelope components on middle ear epithelium.
Differentiation between renal allograft rejection and cyclosporine toxicity: a clinicopathological study. The differentiation between cyclosporine nephrotoxicity (CyN) and acute rejection (AR) still remains a matter of intensive research. In a retrospective study over the last 2 years, we assessed the clinical and histopathological data of 43 episodes of renal dysfunction in 39 renal transplant recipients immunosuppressed with cyclosporine (CyA). Ten episodes (23.2%) were identified as AR and 10 (23.2%) as acute CyN; in six cases (13.9%), signs of both AR and CyN were found. Fever (80%), oliguria (50%), and edema (50%) were prominent features in AR, but not in CyN. Renal blood flow was higher in the nephrotoxicity group and for corresponding degree of renal dysfunction. Significant hyperuricemia (greater than 8 mg/dL) was a prominent finding in CyN (80%) and to a lesser extent in AR (20%). The helper to suppressor cell ratio in the peripheral blood remained stable or slightly decreased in all cases with CyN, but increased in 70% of the cases with AR. CyN was associated with significantly higher whole blood CyA levels (P less than 0.005) and there was a positive correlation between plasma creatinine and CyA levels during the nephrotoxicity episodes (P less than 0.02). Diffuse mononuclear cell infiltrate was observed in 90% of the biopsies with AR and only in 20% with CyN (P less than 0.005). Concerning the extent of the tubular lesions, no significant differences were observed between the two groups. In contrast, vascular lesions such as arterial endothelial proliferation (P less than 0.05), hyalinosis (P less than 0.05), and glomerular capillary thrombi (P less than 0.05) were more commonly seen in nephrotoxicity biopsies.
The paradox of 'chronic unstable angina' secondary to an organizing intracoronary thrombus [published erratum appears in South Med J 1991 Sep;84(9):1074, 1160] We have described a case in which coronary vasospasm and the resultant endothelial shear stresses most likely caused plaque rupture and transient coronary occlusion at the site of a minor atherosclerotic narrowing. Remodeling then led to a long, complex stenotic lesion that included ruptured and dissected endothelium and organized thrombus. This led to severe anginal symptoms over a 3-month period, or the paradox of "chronic unstable angina." Angioplasty was difficult but eventually successful after pulsed high-pressure balloon inflations and intracoronary thrombolytic therapy with streptokinase. This case demonstrates that the clinical course of a patient with minor or insignificant coronary disease may be a dynamic process that cannot be entirely predicted even by recent invasive cardiac studies.
Lhermitte-Duclos disease and Cowden disease: a single phakomatosis. Two unrelated patients with macrocephaly, seizures, and mild cerebellar signs had a dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). Both also had autosomal dominant Cowden disease as evidenced by facial, oral, and acral papules. In the two families, 9 sibs demonstrated the mucocutaneous lesions, thyroid disease, breast tumors, and ovarian tumors compatible with the diagnosis of Cowden disease. Some of the sibs also showed various degrees of neurological signs such as macrocephaly, mental retardation, seizures, tremor, and dysdiadochokinesia. Magnetic resonance imaging scans of sibs of one family demonstrated megalencephaly and other mild abnormalities. The occurrence of these two rare disorders in single patients is more than a coincidence, and the clinical findings in the combined condition establishes it as a new phakomatosis.
Endothelin effects on renal function and tubuloglomerular feedback. Analysis of our data in conjunction with other recent literature allows the following conclusions regarding the role of endothelin in the tubuloglomerular feedback control mechanism and in the pathogenesis of acute ischemic renal failure: (1) Endothelin reduces nephron filtration rate in the nephrons with interrupted signal perception at the macula densa, in accord with preglomerular arteriolar constriction. Yet, the increase in filtration fraction in the whole kidney clearance study suggests a preferential postglomerular arteriolar constriction. Taken together, endothelin, which is a very potent renal vasoconstrictor, seems to constrict both preglomerular and postglomerular arterioles with a predominant constriction of the latter at the doses employed. (2) The endothelin-induced natriuresis is due to a fall in tubular reabsorption, reflecting a direct tubular action, possibly related to an elevation in blood pressure. (3) At the doses of endothelin used and under the present experimental conditions, changes in the magnitude of tubuloglomerular feedback (TGF) response or the feedback characteristic could not be detected. (4) No evidence was found for a participation of endothelin in the pathogenesis of acute postischemic renal failure, as evidenced by the absence of an improvement in glomerular filtration after treatment with endothelin antiserum.
Idiopathic photoreceptor dysfunction causes unexplained visual acuity loss in later adulthood. Progressive visual loss, caused by initially unrecognized retinal photoreceptor dysfunction predominantly affecting cones, developed in six patients in late adulthood. Because of minimal ophthalmoscopic retinal abnormalities, the patients initially had been suspected of having optic neuropathy or psychogenic visual loss. The correct diagnosis was ultimately established by finding electroretinographic abnormalities and color discrimination losses out of proportion to visual acuity loss. In no case was a causative agent or a family history of photoreceptor disease identified. In many other respects, however, features of the patients' illness resemble those described previously under the headings of cone dysfunction syndrome, cone dystrophy, cone degeneration, and cone-rod dystrophy.
Volvulus of the splenic flexure: radiographic features. Six cases of splenic flexure volvulus were studied over a 14-year period. The patients were aged 15-62 years. Five of the six patients were mentally retarded, lifelong residents of a long-term-care institution. Two patients had congenital absence of normal colonic attachments; the other four patients had elongated mesocolons, presumably from chronic constipation. All patients underwent abdominal radiography, followed by a barium enema study. In the appropriate clinical setting, radiographic diagnosis of a splenic flexure volvulus is suggested when the following are seen: (a) a markedly dilated, air-filled colon wtih an abrupt termination at the anatomic splenic flexure; (b) two widely separated air-fluid levels, one in the transverse colon and the other in the cecum; (c) an empty descending and sigmoid colon; and (d) a characteristic beak at the anatomic splenic flexure at a barium enema examination.
Results of surgical therapy for biliary dyskinesia. One hundred eighty-seven patients who presented with symptoms consistent with biliary colic but had no ultrasonic evidence of cholelithiasis were observed in an effort to identify those with a functional gallbladder disorder that might benefit from surgical intervention. All patients underwent quantitative evaluation of gallbladder emptying using cholecystokinin biliary scanning, and ejection fractions less than 35% were considered abnormal. One hundred twenty-nine patients (69%) had abnormal ejection fractions, and 88 (68%) of these subsequently underwent cholecystectomy. Sixty of the surgical specimens revealed pathologic changes. Eighty-four percent of patients successfully contacted for follow-up experienced complete relief, and another 13% had partial relief of preoperative symptoms. Only two patients reported no change in symptom complex. Twenty-nine patients with abnormal ejection fractions elected not to undergo surgery. Fifty-nine percent of these patients continued to experience symptoms of biliary colic at a mean follow-up of 22 months. Of the 44 patients with normal ejection fractions, 35 (80%) reported resolution of symptoms during follow-up of medical treatment. Cholecystokinin biliary scanning can help identify patients with acalculous, functional gallbladder disease who may benefit from cholecystectomy.
Impedance cardiography for repeated determination of stroke volume in elderly hypertensives: correlation with pulsed Doppler echocardiography. In this double-blind, crossover study the authors have validated stroke volume determination by impedance cardiography against the pulsed Doppler echocardiographic method in elderly hypertensives. They found a good correlation between the stroke volume values obtained by the two methods over a range of values from 30 to 130 mL. The coefficient of linear regression was about .95 at each visit. The mean of the differences was -0.73 mL with a standard deviation of 8.46. Given that individual differences are normally distributed, the values corresponding to 2 standard deviations of the mean define a range covering 95% of the observed differences. From the distribution of the data around the mean plot it appears that, in comparison with pulsed Doppler, impedance cardiography tends to slightly underestimate stroke volumes of greater than 90 mL and to overestimate values of less than 50 mL. The results of this study indicate that impedance cardiography may represent a reliable alternative to pulsed Doppler echocardiography for the noninvasive estimation of cardiac output at rest in elderly patients.
Tricuspid annular motion. Triscupid annular motion is related to right ventricular systolic function in the same way mitral annulus motion is related to left ventricular function. Tricuspid annular excursion reflects the longitudinal motion of the right ventricle, and the systolic descent of the anulus correlates with systolic venous inflow to the right atrium. However, it has not been shown clearly how to reproducibly quantify this motion. Therefore we describe a method to measure triscuspid annular motion using two-dimensional oriented M-mode echocardiography from the apical view. We studied a group of 10 normal subjects (mean age, 28.7 years; range, 25 to 38 years) and a group of 29 patients (mean age, 57.2 years; range, 20 to 84 years) with disease of the left side of the heart but no evidence of involvement of the right side of the heart. In each subject, tricuspid and mitral annular motion were measured respectively at their lateral, septal or medial, anterior, and posterior margin points. The total tricuspid annular motion for normal subjects was, as follows: lateral, 24.9 +/- 3.5 mm; medial, 20.1 +/- 2.5 mm; anterior, 21.6 +/- 3.8 mm; and posterior, 22.3 +/- 2.3 mm. Interobserver and intraobserver variability was low, with a coefficient of variance for the different annular points ranging from 6.19% to 11.56% between observers and from 4.10% to 7.26% within observer. We conclude that it is possible to measure tricuspid annular motion with this method in a reproducible way and to use it as a diagnostic tool in evaluating function of the right side of the heart.
Asymptomatic myocardial ischemia during percutaneous transluminal coronary angioplasty and importance of prior Q-wave infarction and diabetes mellitus. Eighty-eight patients undergoing percutaneous transluminal coronary angioplasty (PTCA) of 100 stenoses were studied for the presence of factors deemed significant in the etiology of silent myocardial ischemia. Thirty-two patients were asymptomatic during balloon dilations of 36 arteries, and 56 patients had angina during PTCA of 64 arteries. There were no differences in age, sex, prior anginal history, antianginal regimen, extent of coronary artery disease and number or duration of inflations between the 2 study groups. Previous infarction (33 vs 12%, p less than 0.02), Q waves in the target area (31 vs 7%, p less than 0.005) and diabetes mellitus (36 vs 17%, p less than 0.05) were present more often in the asymptomatic group. Sixty-four% of all asymptomatic patients had either diabetes or previous infarction in the target territory. Collateral circulation was more frequent in asymptomatic patients, probably reflecting the ability of collateral arteries to ameliorate ischemia. During 2-vessel PTCA, patients without angina during dilation of only 1 of the 2 treated arteries (discordant responders) had previous infarction in that artery's territory (5 of 5, 100%), whereas patients without previous infarction were either symptomatic or asymptomatic (concordant responders) during PTCA of both arteries. This study shows that asymptomatic ischemia occurs frequently during PTCA in patients with symptomatic coronary disease. Prior Q-wave infarction and diabetes mellitus are important, independent factors associated with painless ischemia. It is suggested that infarction produces a localized dysfunction of afferent cardiac pain fibers, whereas diabetes can cause a global cardiac sensory neuropathy.
Expanded clinical evaluation of lovastatin (EXCEL) study results: IV. Additional perspectives on the tolerability of lovastatin. This randomized, double-blind, multicenter, diet-and-placebo-controlled study was designed to clarify the dose-response relationship of lovastatin therapy to lipid-modifying efficacy and drug-related adverse events. Exclusion criteria were minimized so that study patients were representative of the majority of patients with moderate hypercholesterolemia seen in medical practice. After 6 weeks on the American Heart Association Step 1 Diet, a total of 8,245 patients were randomly assigned to 48 weeks of treatment with diet and placebo or lovastatin at dosages of 20 or 40 mg once a day or 20 or 40 mg twice a day. All adverse events were monitored, with particular attention to evaluation of liver and muscle. Liver transaminase elevations suggestive of possible hepatotoxicity, defined as successive elevations in either aspartate transaminase or alanine aminotransferase greater than 3 times the upper limit of normal, occurred in equal numbers of placebo and lovastatin 20 mg/day treated patients (0.1%). The frequencies were higher in lovastatin 40 mg/day and 80 mg/day patient groups (0.9 and 1.5%, respectively). No patient was diagnosed as having clinically symptomatic hepatic dysfunction. Creatinine kinase (CK) elevations above the upper limit of normal occurred frequently in placebo- (29%), as well as lovastatin-treated patients (29-35%), and muscle symptoms were reported with similar frequency in all groups (7-9%). The combination of muscle symptoms with marked CK elevations (greater than 10 times the upper limit of normal) was seen in only five patients: one in a 40 mg/day dose group and four in the 80 mg/day dose group. No patient developed rhabdomyolysis. The incidence of clinical and laboratory adverse events requiring discontinuation was 6% for the placebo group and from 7% (20 mg/day) to 9% (80 mg/day) for lovastatin treatment groups. No new types of adverse experiences related to lovastatin treatment were reported. Lovastatin, as an adjunct to diet for the reduction of elevated LDL cholesterol, was generally very well tolerated.
Cis-platinum chemotherapy for ocular basal cell carcinoma. We have used intravenous cis-platinum chemotherapy in the treatment of three patients with basal cell carcinoma of the lid extending into the orbit. Cis-platinum chemotherapy caused a reduction in tumour size and thereby delayed surgery in all cases. It allowed for local resection in one case, appeared to delay a patient's exenteration in a second case, and was used prior to radiotherapy in a third case. While not curative, cis-platinum may be useful as an adjuvant to decrease tumour mass prior to local excision and for patients who refuse or must delay exenteration.
Percutaneous radiofrequency catheter ablation for supraventricular arrhythmias in children. Nineteen procedures were performed in 17 children, aged 10 months to 17 years, using catheter radiofrequency applications for the management of malignant or drug-resistant supraventricular tachyarrhythmias. Diagnoses were junctional ectopic tachycardia in 1 patient, atrioventricular (AV) node reentrant tachycardia in 4 and accessory pathway-mediated tachycardia in 12. Accessory pathway locations were left lateral (n = 4), posteroseptal (n = 3), left posterior (n = 2), right posterolateral (n = 1), right posterior paraseptal (n = 1), right intermediate septal (n = 1) and right anterior (n = 1). Ablation of accessory pathways was performed using 20 to 40 W of energy. The catheter was passed retrograde to the left ventricle in patients with a left-sided pathway and anterograde to the right atrium in those with a right-sided or posteroseptal pathway. In the 12 patients with an accessory pathway, radiofrequency applications were successful in 11 pathways and failed in 2. There were no recurrences of accessory pathway-mediated tachycardia. Atrioventricular node reentrant tachycardia was treated by AV node modification using 15 W of energy applied until first degree AV block occurred. After radiofrequency catheter ablation, there was a prolonged AH interval, tachycardia was not inducible and tachycardia recurred in one patient. For the patient with junctional ectopic tachycardia, 15 to 18 W of energy was delivered at the site of the maximal His bundle electrogram until sinus rhythm and normal AV conduction appeared. After a recurrence, a second procedure abolished tachycardia and AV conduction. In summary, radiofrequency catheter ablation was initially successful in 17 of 19 procedures and ultimately curative in 14 (82%) of 17 patients with no serious complications. Radiofrequency catheter ablation appears to be a safe and effective method for the management of supraventricular tachyarrhythmias in children.