shukoor65/lab-rag-model huggingface dataset:
Elevation of serum interleukin-6 concentration precedes acute-phase response and reflects severity in acute pancreatitis. Experimental studies have shown that interleukin-6 induces all major acute-phase proteins in the liver, including C-reactive protein. In 50 patients with acute pancreatitis, the serum concentrations of interleukin-6 and C-reactive protein were determined daily during the first week of hospitalization. Patients were divided into three groups according to clinical criteria: mild pancreatitis (less than or equal to 1 complication; n = 25), severe pancreatitis (greater than or equal to 2 complications; n = 15), and lethal outcome (n = 10). Patients with mild disease showed initially slightly elevated levels of interleukin-6 (22.0 +/- 9.8 U/mL) that decreased to low levels within 4 days (5.0 +/- 1.0 U/mL). In patients with severe pancreatitis, serum concentrations of interleukin-6 were initially clearly elevated (35.0 +/- 7.5 U/mL) and remained slightly elevated until day 7 (13.0 +/- 2.0 U/mL). Patients with lethal outcome had markedly elevated initial interleukin-6 concentrations (61.0 +/- 15.0 U/mL) that decreased but were still elevated at day 7 (26.0 +/- 2.5 U/mL). In all three groups, C-reactive protein concentrations followed the course of interleukin-6 concentrations by 1 day. There was a positive correlation between maximal interleukin 6 concentrations and maximal increases in the serum concentrations of C-reactive protein (r = 0.66). At days 1 and 2, increased (greater than 15 U/mL) interleukin-6 concentrations (positive predictive value, 91%; negative predictive value, 82%) predicted a severe or lethal course of the disease more accurately than elevated [greater than 0.10 g/L (greater than 10 mg/dL)] C-reactive protein concentrations (positive predictive value, 67%; negative predictive value, 79%). In conclusion, elevated serum concentrations of interleukin-6 followed by increased levels of C-reactive protein reflect the severity of acute pancreatitis.
Acquired aphasia, dementia, and behavior disorder with epilepsy and continuous spike and waves during sleep in a child. Severe persistent neuropsychological disorders sometimes develop in the course of a focal epilepsy of unknown origin in previously normal children. Very frequent bilateral focal or generalized discharges are often noted on the sleep EEG records of these patients with no evidence of clinical seizures. The relation between these paroxysms and the observed deterioration remains unclear. We report a child with a partial complex epilepsy and severe disturbances of language, cognition, and behavior acquired in the early years of development who was followed for 15 years. A correlation between the evolution of the striking EEG abnormalities during sleep and the neuropsychological disorders could be established retrospectively. The observed sequence of onset and recovery of the aphasia, the dementia, and the "psychotic" behavior makes a direct causal relation between the deficits quite unlikely. Rather it suggests an association of independent symptoms with a specific language disorder becoming manifest in the course of the evolution. This child shows many of the main characteristics of the syndromes of "acquired aphasia with convulsive disorder" (Landau-Kleffner syndrome) and "epilepsy with continuous spike waves during sleep." Both syndromes describe probably different facets of a similar underlying, still unexplained cerebral dysfunction.
Optimal dose of lignocaine for preventing pain on injection of propofol. The purpose of this study was to define the optimum dose of lignocaine required to reduce pain on injection of propofol. We conducted a prospective, randomized, double-blind trial on 310 patients undergoing anaesthesia. Patients were allocated to four groups according to the lignocaine dosage: group A (control), no lignocaine; group B, lignocaine 0.1 mg kg-1; group C, lignocaine 0.2 mg kg-1; group D, lignocaine 0.4 mg kg-1. Our results showed that a dose of lignocaine 0.1 mg kg-1 significantly reduced the incidence of pain and that there was no improvement when the dose was increased.
Valvular heart disease: management and intervention. Clinical overview and discussion. The diagnosis and estimation of severity of valvular disease can usually be made by a well done history and physical examination. We now have a wealth of noninvasive techniques capable of imaging the heart and the great vessels. With Doppler echocardiography and magnetic resonance imaging we are beginning to develop methods that can measure pressures and flow. All of these advances have duplicated to a large extent the information obtainable in the past only from cardiac catheterization and angiography. In some instances, in aortic and mitral stenosis, for example, echo-Doppler evaluation is so good that catheterization is rendered unnecessary. The decision to send patients to surgery without catheterization is especially secure when the valvular obstruction is very severe. When the gradient is only moderately increased, then estimation of severity depends on flow, and in many cases these patients are sent to catheterization before surgery is recommended. With regurgitant lesions there is less confidence with noninvasive techniques that the severity is always correctly estimated, and therefore catheterization and angiography are more often necessary before sending the patient to surgery. At the present time cardiac catheterization and angiography are required 1) whenever there is an inconsistency between the clinical picture and noninvasive information, for example, the symptomatic patient with aortic stenosis who has mild-to-moderate aortic stenosis by echo-Doppler techniques, and 2) whenever additional information not obtainable by noninvasive techniques is necessary, for example, the need to know the status of the coronary arterial circulation. Noninvasive techniques have not in any way replaced a good history and physical examination.
Symptomatic xanthogranuloma of choroid plexus with unilateral hydrocephalus. Case report. Xanthogranulomas involving the choroid plexus of the lateral ventricles are generally asymptomatic lesions. The case is reported of a 50-year-old man in whom a xanthogranuloma of the choroid plexus had occluded the left trigone, causing unilateral hydrocephalus of the left temporal horn and neurological symptoms. A review of the literature shows that xanthogranulomas of the glomus of the lateral ventricles differ from the xanthomatous cystic lesions of the third ventricle, which are probably akin to colloid cysts.
Inherited protein C deficiency and nonhemorrhagic arterial stroke in young adults. Out of a consecutive series of 50 young people less than 45 years old with nonhemorrhagic arterial stroke, three patients had inherited protein C deficiency. CT revealed hypodense areas consistent with the clinical picture, and angiography showed occlusion of some intracranial arterial vessels. Other possible associated causes of stroke were ruled out. One patient had a transient ischemic attack and a peripheral venous thrombosis prior to the actual stroke, whereas the others were completely asymptomatic, as were relatives with the same deficiency. We suggest determining protein C in ischemic stroke of all young adults, especially when major risk factors are excluded.
Problems in the surgical treatment of interstitial cystitis. Two series of patients with histologically proven interstitial cystitis that was unresponsive to hydrostatic bladder distension and intravesical chemotherapy with dimethyl sulfoxide have been studied. In the first series 24 patients were treated by subtotal cystectomy and substitution cystoplasty without further consideration; 8 of these 24 patients had persistent frequency due to active disease in the remaining trigone and/or urethra and in 2 cases this was severe. Because of this experience the second group of patients had routine biopsy of the trigone and assessment of urethral sensation as part of the initial assessment. In those in whom the trigone was unaffected, treatment was unchanged. If the trigone was affected, total cystourethrectomy was performed with substitution cystourethroplasty unless the patient chose or was advised to avoid surgery altogether or to have a simpler option such as conduit or continent urinary diversion. Trigonal biopsies should be part of the routine assessment of all patients being considered for surgery, since residual active disease is a major cause of dissatisfaction after subtotal cystectomy and substitution cystoplasty.
Radiotherapy improves the outlook for patients older than 1 year with Pediatric Oncology Group stage C neuroblastoma. Children older than 1 year of age who have neuroblastoma with complete or partial removal of the primary tumor and positive intracavitary lymph nodes (Pediatric Oncology Group [POG] stage C) are a small but higher-risk subset of patients. To further evaluate the importance of identifying patients with POG stage C neuroblastoma and to assess the efficacy and toxicity of adding concurrent radiation therapy (RT) to chemotherapy (CT) in these children, a randomized study was conducted. Eligible patients received cyclophosphamide 150 mg/m2 orally days 1 to 7 and Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH) 35 mg/m2 intravenously (IV) on day 8 (CYC/ADR) every 3 weeks for five courses with or without RT to primary tumor and regional lymph nodes (24 to 30 Gy/16 to 20 fractions). Second-look surgery was advised to evaluate response and to remove residual disease. Continuation therapy alternated CYC/ADR every 3 weeks with cisplatin 90 mg/m2 day 1 followed by teniposide 100 mg/m2 day 3 (CDP/VM) for two courses each. Secondary CT with CDP/VM alone was available for patients not achieving complete response (CR) following induction treatment and second-look surgery. Of 29 eligible patients randomized to CT alone, 13 achieved CR, and nine are disease-free (NED) 1 to 52 months (median, 35 months) off therapy. Twenty-two of 33 eligible cases treated with CT/RT attained CR, and 19 are NED 1 to 77 months (median, 23 months) off therapy. Local and metastatic relapses occurred in both arms. Differences in CR, event-free survival, and survival rates were significant, P = .013, .009, and .008, respectively. Surgical compliance was excellent and complications uncommon. Therapy was tolerable in both groups but hematopoietic toxicity was more common in the CT/RT arm. We conclude that POG stage C neuroblastoma in children older than 1 year of age is a higher-risk group that should be identified, that CT/RT provides superior initial and long-term disease control compared with CT alone in this patient subset, and that the occurrence of metastatic failures in both treatment groups suggests a need for more aggressive chemotherapy.
Pulmonary origin of left anterior descending coronary artery in tetralogy of Fallot. We report the case of a 4-year-old girl with an anomalous origin of the left anterior descending coronary artery from the pulmonary artery in association with tetralogy of Fallot. Creation of an intrapulmonary tunnel from a newly created aortopulmonary window to the coronary ostium in the pulmonary trunk and total repair of tetralogy of Fallot were successfully carried out. The advantage of intrapulmonary tunnel technique (Takeuchi method) for this very rare combination is stressed.
Operative survival and 40 year follow up of surgical repair of aortic coarctation. OBJECTIVE--To study early and late mortality after surgical correction of coarctation of the aorta. DESIGN--Data on 223 patients operated on at the Westminster Hospital, London, between 1946 and 1981, were collected and updated by questionnaire. PARTICIPANTS--All 223 patients recorded as undergoing operation for aortic coarctation up to the end of 1981. Fifteen of 197 survivors were lost to follow up; most of them were patients from overseas. OUTCOME AND RESULTS--The early mortality (within one month of operation) was 12% overall, 2.6% for elective surgery, and 0% for the 77 patients undergoing surgery since 1968. Survivors were followed up for a total of 3288 patient years; in 27 follow up lasted more than 30 years. In a few it reached 40 years. Twenty two patients died during this period, 18 from causes that could be attributed to coarctation or its repair. Mortality was highest more than 20 years after the operation. CONCLUSION--Repair increased life expectancy in patients with aortic coarctation. Late problems caused by persistent hypertension or recoarctation became apparent in long term survivors. The increased risk of late mortality associated with the duration of preoperative hypertension was not statistically significant. There were no deaths from cerebrovascular accidents. (In an earlier necropsy series cerebrovascular accidents accounted for 11.8% of deaths.) The incidence of deaths from aneurysms resembled that in the earlier necropsy series.
Pneumoparotid: a case report. Pneumoparotid is an unusual condition resulting from air being forced into the parotid ductal system. When it develops, it is a result of increased intraoral air pressure. Diagnosis is based on the etiologic factor, palpatory evidence of tissue emphysema, the escape of frothy saliva from the involved duct, and the sialographic finding of retained ductal air and ductal infection.
Vascular trauma as a result of therapeutic procedures for the treatment of malignancy. Twenty-two iatrogenic vascular injuries caused by resection of tumors with local recurrences or adjuvant chemotherapy were treated over a 42-month period in 11 female and 8 male patients with cancer (58% had documented metastases). Sixteen of 22 vascular injuries were caused by intraarterial administration of chemotherapy (8 emboli; 8 direct catheter trauma), and six were caused by resection of tumors with local invasion. The injuries were extremity ischemia (15 extremities in 13 patients), pseudoaneurysm (4), expanding hematoma (3 injuries in 2 patients). Twenty-two surgical repairs included bypass of severely damaged arteries in five, embolectomy in five, interposition grafts in three, pseudoaneurysm resection and primary repair in three, primary repair of three vascular injuries, and one patch angioplasty. Leg amputation was required in two patients. Seventeen of 19 patients had successful vascular repairs with resolution of preoperative indications for vascular repair without intraoperative or postoperative deaths. Long term follow-up (mean, 17 months) showed no recurrence of vascular problems; however, two patients died of metastatic cancer at 6 and 24 weeks after vascular repair. This study supports an aggressive approach to the management of vascular injuries caused by therapeutic interventions for malignancy despite the presence of metastatic disease.
Abnormal guanine nucleotide regulatory protein in MVP dysautonomia: evidence from reconstitution of Gs. We and others have used the term MVP dysautonomia for a particular subset of hyperadrenergic dysautonomia patients. The role of the stimulatory guanine nucleotide regulatory protein (Gs) in this dysautonomia was studied by cholate extraction of Gs from erythrocytes from 11 normal subjects and 14 symptomatic dysautonomic patients and reconstitution into cyc-S49 lymphoma membranes, which have normal receptor and adenylyl cyclase but lack Gs. Isoproterenol-stimulated adenylyl cyclase activity in the dysautonomia group was increased compared to that in controls [3.66 +/- 0.20 (mean +/- SE; n = 14) vs. 2.87 +/- 0.14 (n = 11) U cyc- reconstituted activity/mg erythrocyte protein; P less than 0.05]. beta-Adrenergic receptor high affinity state formation was greatest in the severely symptomatic group [KL/KH: severe symptoms, 130 +/- 48 (n = 6); mild symptoms, 33 +/- 7 (n = 7); control, 27 +/- 6 (n = 11); severe dysautonomia distinct, P less than 0.017]. Sodium dodecyl sulfate-polyacrylamide gels of cholera toxin-dependent ADP-ribosylated G-proteins yielded no gross distinction between severely symptomatic and control groups. This subset of hyperadrenergic dysautonomia patients, thus, has supercoupled beta 2-adrenergic receptors (increase in both agonist binding and cyclase activation) conferred by an abnormal Gs, whose effects on agonist binding reflect the severity of illness.
Ischemic complications of abdominal aortic surgery. From 1982 through 1988, 634 consecutive patients underwent abdominal aortic reconstruction for occlusive (37%) or aneurysmal (63%) disease. We studied the ischemic problems affecting the branches of the aorta, excluding the coeliac and superior mesenteric arteries. Ischemic colitis (0.6%), spinal cord ischemia (0.16%), renal insufficiency (17%), and lower limbs ischemia (6.5%) were the major problems encountered. We identified the most significant factors associated with these complications such as hypotension, emergency, hypovolemia, preoperative renal function, suprarenal clamping, the quality of the preoperative investigation, and have suggested some specific preventive measures.
Asbestos induced pericardial effusion and constrictive pericarditis. The number of disorders attributable to asbestos exposure has increased gradually over the years. The latest to be recorded is pericardial effusion and constrictive pericarditis, and three cases are reported here. A man with bilateral pleural thickening and plaques developed acute pericarditis and an effusion and was treated by pericardiectomy. Two men died from constrictive pericarditis associated with bilateral pleural effusions and diffusion pleural thickening. The pericardium showed nonspecific fibrous thickening. All had been occupationally exposed to asbestos. In the fatal cases the lungs contained amphibole fibres, in keeping with a modest degree of occupational exposure. Asbestos produces progressive fibrosis of the pericardium that is similar to diffuse pleural thickening and may be fatal. Both conditions may develop after relatively short or light exposure.
The phenotype of the X-linked dystonia-parkinsonism syndrome. An assessment of 42 cases in the Philippines. The clinical phenotype of X-linked recessive torsion dystonia was documented in 42 affected individuals from 21 families. In 7 families, there were 9 sibships (core families) with 2 or more affected individuals available for evaluation. The ages of the patients ranged from 29 to 79 years with a mean of 46.2 +/- 10.1 years; the mean age of onset of dystonia was 35.0 +/- 8.0 years with a range of 12 to 48 years; and the mean duration of illness was 11.1 +/- 7.9 years. First manifestations were noted in the lower extremities in 36%, the axial musculature in 29%, the upper extremities in 23%, and in the head in 12% of the cases. The majority of patients displayed gait abnormalities (90%), leg dystonia (79%), oromandibular dystonia (64%), neck dystonia (57%), blepharospasm (57%), and truncal dystonia (52%). The disease generalized in 90% of the cases within 1 to 11 years of onset (median duration, 5 years). Overall, the condition was disabling, but the Fahn-Marsden disability score did not correlate with age of onset, duration of illness, site of onset, rate of generalization, or presence of parkinsonism. Thirty-six percent of the cases displayed at least 1 of the following "parkinsonian symptoms": bradykinesia, tremor, rigidity, loss of postural reflexes and a shuffling gait. Parkinsonism was diagnosed as definite in 14%, probable in 2%, and possible in 19% of the cases. Given this high association of dystonia and parkinsonism, we propose to call the disorder X-linked dystonia-parkinsonism syndrome (XDP).
Computer- and robot-assisted resection of thalamic astrocytomas in children. Six children ranging in age from 2 to 10 years who harbored deep benign astrocytomas were operated upon using a computer- and robot-assisted system. A radical excision was achieved in all cases with no significant morbidity nor any mortality. The system consists of an interactive, three-dimensional display of computed tomographic image contours and digitized cerebral angiograms taken using the Brown-Roberts-Wells stereotactic frame. The surgical retractor is held and manipulated using a PUMA 200 robot. The position and orientation of the surgical retractor is displayed on the three-dimensional display. Preoperative planning and simulation are important features of this system. Movement of the brain after removal of the tumor and cerebrospinal fluid is substantial, so the tumor removal is based on visually defined margins. Enhanced computer graphics and robotic devices are important adjuncts to neurosurgical procedures and will find increasing use in the future.
Randomized trial of doxorubicin, bisantrene, and mitoxantrone in advanced breast cancer: a Southwest Oncology Group study. Four hundred eleven women with metastatic breast cancer were randomly assigned to receive either 60 mg/m2 doxorubicin (130 patients), 320 mg/m2 bisantrene (146 patients), or 14 mg/m2 mitoxantrone (135 patients). The doses were given intravenously every 3 weeks with a cross-over design to determine their relative efficacy and toxicity. To be eligible, patients must have had one previous chemotherapy regimen, and patients who were estrogen receptor positive must have failed endocrine therapy. There were 365 patients assessable for response and 399 assessable for toxic effects. The median age was 57 years; 18% were premenopausal or perimenopausal. Visceral dominant disease was present in 66% of the patients. Ninety-seven percent of the patients had a disease-free interval from diagnosis to first recurrence of less than 1 year. The response rate was 28% with doxorubicin, 13% with bisantrene, and 14% with mitoxantrone (P = .004). Median time to treatment failure was 133 days with doxorubicin, 66 days with bisantrene, and 68 days with mitoxantrone (logrank P = .06). The median survival was 315 days for doxorubicin, 290 days for bisantrene, and 177 days for mitoxantrone (logrank P = .04), although survival at 2 years was similar for all three agents. There were five responses in the 66 patients crossed over to doxorubicin and one response each for patients crossed over to bisantrene (39 patients) or mitoxantrone (63 patients). Toxicity leading to discontinuance of therapy was more common with doxorubicin, and discontinuance of therapy was due primarily to patient's request or cardiotoxicity. The major dose-limiting toxic effect for all three agents was leukopenia. Nausea and vomiting, mucositis, and alopecia were more severe with doxorubicin. Congestive heart failure developed in nine patients treated with doxorubicin, zero patients treated with bisantrene, and two patients treated with mitoxantrone. A decrease in the left ventricular ejection fraction, as defined by moderate to severe Alexander grade changes, was more common in patients treated with doxorubicin (doxorubicin-treated patients = 20%, bisantrene-treated patients = 5%, and mitoxantrone-treated patients = 10%). This study demonstrates that bisantrene and mitoxantrone have only modest activity in metastatic breast carcinoma. The activity of doxorubicin is greater than that of the other two agents, but at a cost of increased toxicity.