123rc/medical_text huggingface dataset:
Hypoglycemia, hypotonia, and cardiomyopathy: the evolving clinical picture of long-chain acyl-CoA dehydrogenase deficiency. Inherited defects in fatty acid oxidation, which have been described and diagnosed with increasing frequency in the last decade, are most commonly attributed to a deficiency in the activity of medium-chain acyl-CoA dehydrogenase. Few cases of the related enzyme defect of long-chain acyl-CoA dehydrogenase activity have been reported. An infant with documented long-chain acyl-CoA dehydrogenase deficiency is described with a detailed metabolic profile, long-term clinical follow-up, and response to treatment. This patient is compared with the seven previously published cases of this disorder in order to stress the unique features of the initial presentation, more subtle late manifestations of the disease, and clinical and biochemical differentiation from the more common medium-chain acyl-CoA dehydrogenase deficiency. This report stresses the enlarging spectrum of the clinical presentation and natural history of this defect in fatty acid oxidation.
Skin reflectance pulse oximetry: in vivo measurements from the forearm and calf. This study describes the results from a series of human experiments demonstrating the ability to measure arterial hemoglobin oxygen saturation (SaO2) from the forearm and calf using a reflectance pulse oximeter sensor. A special optical reflectance sensor that includes a heating element was interfaced to a temperature controller and a commercial Data-scope ACCUSAT pulse oximeter that was adapted for this study to perform as a reflectance pulse oximeter. The reflectance pulse oximeter sensor was evaluated in a group of 10 healthy adult volunteers during steady-state hypoxia. Hypoxia was induced by gradually lowering the inspired fraction of oxygen in the breathing gas mixture from 100 to 12%. Simultaneous SaO2 measurements obtained from the forearm and calf with two identical reflectance pulse oximeters were compared with SaO2 values measured by a finger sensor that was interfaced to a standard Datascope ACCUSAT transmittance pulse oximeter. The equations for the best-fitted linear regression lines between the percent reflectance, SpO2(r), and transmittance, SpO2(t), values in the range between 73 and 100% were SpO2(r) = -7.06 + 1.09 SpO2(t) for the forearm (n = 91, r = 0.95) and SpO2(r) = 7.78 + 0.93 SpO2(t) for the calf (n = 93, r = 0.88). The regression analysis of the forearm data revealed a mean +/- SD error of 2.47 +/- 1.66% (SaO2 = 90-100%), 2.35 +/- 2.45% (SaO2 = 80-89%), and 2.42 +/- 1.20% (SaO2 = 70-79%). The corresponding regression analysis of the calf data revealed a mean +/- SD error of 3.36 +/- 3.06% (SaO2 = 90-100%), 3.45 +/- 4.12% (SaO2 = 80-89%), and 2.97 +/- 2.75% (SaO2 = 70-79%).
Placebo controlled trial of xamoterol versus digoxin in chronic atrial fibrillation. Thirteen patients in chronic atrial fibrillation with a normal resting heart rate but with exercise tachycardia and episodes of bradycardia were randomised to treatment periods of two weeks on xamoterol (200 mg twice daily), low dose digoxin, or placebo, in a blind crossover study. The results (mean SEM) of symptom scores, a treadmill exercise test, and 24 hour ambulatory electrocardiographic monitoring were obtained. Xamoterol improved symptom scores and controlled exercise heart rate better than digoxin. Xamoterol was better than digoxin or placebo in reducing the heart rate response to exercise and tended to improve exercise duration. Xamoterol, by reducing the daytime maximum hourly heart rate and increasing the night time minimum hourly heart rate, significantly reduced the difference between the two compared with placebo. In contrast, digoxin tended to reduce both the maximum and minimum hourly heart rates through day and night. Both the frequency and duration of ventricular pauses were reduced by xamoterol but tended to increase with digoxin. Xamoterol reduced both the circadian variation in ventricular response to atrial fibrillation and exercise tachycardia by modulating the heart rate according to the prevailing level of sympathetic activity. These changes were translated into symptomatic benefit for the patients studied.
Beneficial effect of atrial pacing in severe acute aortic regurgitation and role of M-mode echocardiography in determining the optimal pacing interval. The effect of atrial pacing on cardiac performance was assessed in 11 men (aged 20 to 64 years) with recent-onset severe aortic regurgitation (AR), all of whom had diastolic closure of the mitral valve on the echocardiogram. Thermodilution cardiac outputs were determined, and aortic, left ventricular and pulmonary arterial wedge pressures recorded. Once baseline recordings were completed, the pacing rate was increased by increments of 10 beats/min (70, 80, 90...) to a maximal rate of 140 beats/min. The optimal pacing interval, obtained from hemodynamic data, was defined as that at which the lowest filling pressure was associated with the highest cardiac index. This was then compared with a pacing interval derived from the R wave of the electrocardiogram to the diastolic mitral closing point on the M-mode echocardiogram. Such an interval would shorten diastole without affecting forward mitral flow. Atrial pacing improved the overall hemodynamic state in all patients; the most favorable hemodynamics were achieved at heart rates between 110 and 130 beats/min (mean: 120 +/- 8). At the optimal rate, left ventricular end-diastolic pressure decreased from 46 +/- 7 to 23 +/- 12 mm Hg (p less than 0.001), and the pulmonary arterial wedge pressure from 28 +/- 8 to 16 +/- 7 mm Hg (p less than 0.001), while the cardiac index increased from 2.34 +/- 0.46 to 2.63 +/- 0.49 liters/min/m2 (p less than 0.01). The mean difference between the optimal pacing interval determined from the hemodynamic data and the interval derived from the echocardiogram was 18 +/- 21 ms.
Vertical partial laryngectomy: a critical analysis of local recurrence. The purpose of this study was to evaluate local recurrence following vertical partial laryngectomies in 416 patients with either T1N0M0 or T2N0M0 glottic carcinoma. Local failure was reported according to the T stage, the precise tumor location within each stage, the true vocal cord mobility, and the surgical procedure performed. No local recurrences were observed among 42 patients who underwent thyrotomy and cordectomy when the tumor was confined to the middle third of the mobile true vocal cord. Local failure occurred in 8 of 111 (7.2%) patients in whom hemilaryngectomy was performed for tumors confined to one mobile true vocal cord. There was a diverse group of lesions within each T stage that responded differently to the surgical approaches. The differences in the initial recurrence rates are discussed in terms of careful preoperative assessment and choice of surgical technique for early glottic carcinoma.
Correlation between bromodeoxyuridine-labeling indices and patient prognosis in cerebral astrocytic tumors of adults. Bromodeoxyuridine (BUdR), a nonradioactive thymidine analogue, is taken up by cells in S-phase, and the ratio of BUdR-positive nuclei to the total number of cells counted is defined as the labeling index (LI). In this study, BUdR LI and the clinical course of 50 cerebral astrocytic tumors in adults were analyzed. The obtained LI distributed continuously in a broad range from 0% to 19%. The mean LI of 28 glioblastomas, 12 anaplastic astrocytomas, and ten astrocytomas were 8.5%, 4.2%, and 1.2%, respectively, and these differences were statistically significant (P = 0.05). In the analysis of LI and the recurrence-free period (RFP), regardless of the histologic findings, 23 patients with LI more than 5% had a median RFP of 9.0 months; the median RFP of nine patients with LI of 3% to 5% was 14.7 months. Nine of 13 patients with LI less than 3% have not yet recurred after a median follow-up of 36 months. These differences were also statistically significant by the generalized Wilcoxon test (P = 0.05). The proliferative potential reflected by the BUdR LI is a good clinical indicator for predicting the rate of tumor growth in cerebral astrocytic tumors. In combination with histologic diagnosis, BUdR LI could help in determining a patient's prognosis more precisely.
The role of sphincteroplasty for fecal incontinence reevaluated: a prospective physiologic and functional review. Sixteen female patients (mean age 54.1 years; range 34-74 years) with a 9.8-year (range 1-25 years) history of incontinence to solid stool underwent overlapping sphincteroplasty with internal sphincter imbrication without fecal diversion. All patients were prospectively evaluated with preoperative anorectal manometry, electromyography, and pudendal nerve motor latency assessment, postoperative anorectal manometry, and preoperative and postoperative functional evaluation. Mean and maximal resting pressures increased from 30 mm Hg and 49 mm Hg preoperatively to 40 mm Hg and 57 mm Hg, respectively, postoperatively. Likewise, mean and maximal squeeze pressures increased from 27 mm Hg and 48 mm Hg preoperatively to 39 mm Hg and 73 mm Hg, respectively, postoperatively (P less than 0.01). Furthermore, anal canal high pressure zone length was increased by sphincteroplasty from a mean of 0.9 cm (range 0-3 cm) to a mean of 2.1 cm (range 1-4 cm). These objective physiologic improvements correlated well with subjective functional improvement. Subjectively, functional outcome was rated by patients as excellent in 38 percent, good in 38 percent, fair in 19 percent, and poor in only 5 percent of cases. Overlapping sphincteroplasty with internal sphincter imbrication improves both the anal sphincter physiologic profile and fecal continence.
Effects of tracheal irritation and hypercapnia on tracheal smooth muscle in humans. Both hypercapnia and tracheal irritation are known to constrict the airways in animals. To see whether similar responses occur in humans, we investigated tracheal smooth muscle (TSM) responses to hypercapnia and tracheal irritation with water in 14 paralyzed and anesthetized humans. TSM tone was monitored by measuring the pressure in the saline-filled cuff of the endotracheal tube. Although, tracheal irritation caused TSM constriction in 10 of 14 patients, 4 patients showed no TSM response. Administration of intravenous atropine attenuated the TSM constriction response. Hypercapnia did not cause any change in TSM tone in any of the 14 patients. These results indicate that in paralyzed and anesthetized humans, there exist interindividual differences in the TSM responses to tracheal irritation and that hypercapnia cannot be an effective stimulus for the TSM constriction.
Outcome of patients with chronic affective disorder: a five-year follow-up. Patients with major depression, mania, or schizo-affective disorder that had been present without remission for 2 years or more at intake (N = 129) were followed prospectively for 5 years, as were 580 patients who had been ill for shorter periods at intake. Despite very substantial durations of episode, three-quarters of the chronic patients recovered, although recovery occurred much later in the follow-up period than it did among the nonchronic patients. Factors associated with recovery were less severe illness at intake, lack of psychotic features, good friendship patterns in adolescence, and, most important, a relatively high maximum level of functioning in the 5 years preceding intake.
Stunned myocardium during extracorporeal membrane oxygenation. The "stunned myocardium" is a syndrome of reversible myocardial dysfunction that may be mediated by oxygen-derived free radicals. This phenomenon has been seen in some neonates undergoing extracorporeal membrane oxygenation. We performed echocardiograms and measured creatine phosphokinase isoenzymes and lipid peroxide levels in 16 neonates before, during, and after extracorporeal membrane oxygenation. Infants who developed stunned myocardia by echocardiography did so shortly after initiation of bypass and exhibited concurrent elevations of the MB fraction of creatine phosphokinase. Lipid peroxide levels did not simultaneously rise. These data suggest that oxygen-derived free radicals may not cause the stunned myocardium seen in neonates undergoing extracorporeal membrane oxygenation.
A randomized, controlled trial of the use of music during laceration repair. STUDY OBJECTIVES: To determine whether music significantly reduces the pain and anxiety associated with laceration repair in the emergency department. PARTICIPANTS AND SETTING: Adult patients presenting to the ED at an urban teaching hospital for laceration repair. Exclusion criteria included patients less than 18 years old, having received analgesics, with suspected alcohol or substance intoxication, and in whom laceration repair was complicated by treatment of a more serious medical condition. DESIGN AND INTERVENTIONS: This was a randomized, controlled trial. After giving informed consent, patients were randomly assigned to receive standard laceration repair without music (control) or standard laceration repair with music. Patients assigned to the music group chose an audio tape from 50 available styles and artists. Patients received the music through a headset, and they controlled the volume. Intradermal lidocaine was used in all patients. Data were collected on heart rate, blood pressure, and respirations just before and immediately after wound repair. Psychological variables included the state subscale of the Spielberger State Trait Anxiety Inventory (STAI), a visual analog pain scale rating, and a brief questionnaire. STAI and pain scale ratings were analyzed with Wilcoxon's rank-sum test with an alpha error of .05. RESULTS: Thirty-eight patients (19 per group) completed the protocol. Pain scores were significantly (P less than .05) lower in the music group (mean, 2.09) than in controls (mean, 3.31). Anxiety after the procedure was reduced in both groups, but STAI reduction scores were not significantly different between groups (music, 17.7; control, 18.5). Seventeen of 19 patients (89%) rated music "very beneficial," and 100% said they would use music again. CONCLUSION: Music provides a safe, inexpensive, and effective adjunct for the management of pain in the ED but does not significantly affect anxiety.
Technetium-99m sestamibi in chronic coronary artery disease: the European experience. Since the introduction of technetium-99m methoxy-isobutyl isonitrile (Tc-99m sestamibi) in Europe, there has been a growing interest in its use. Several European multicenter trials have been conducted to evaluate this new agent in relation to the traditional perfusion marker thallium-201, and other studies are in progress to understand the use of this perfusion marker for the diagnosis of coronary disease, for use in conjunction with pharmacologic vasodilation, for use in the assessment of ventricular function and wall motion and for the assessment of interventions.
Toxoplasmosis of the conus medullaris in a patient with hemophilia A-associated AIDS. Case report. Toxoplasma gondii has been reported to be the most common cause of focal brain lesions in patients with acquired immunodeficiency syndrome (AIDS). A case of intramedullary toxoplasmosis of the conus medullaris is reported in a patient with hemophilia A-associated AIDS. The diagnosis is discussed, with particular emphasis on the magnetic resonance imaging appearance.
Prevalence of ulcerative colitis in the Israeli kibbutz population. We carried out an epidemiological study of ulcerative colitis in 279 Israeli kibbutzim (121,403 population). The prevalence on December 31, 1987 was 121.08 per 100,000 population. When the data were stratified according to ethnic groups, the highest point prevalence was found in Israeli-born Jews (220.56 per 100,000 population), more than in Asian-African-born, or European-American-born kibbutz members (139.20 and 78.73 cases per 100,000 population, respectively). There were 68 men and 78 women (ratio of 0.87). The average age of the patients in the year of this survey was 46 years; it was 35 years at the time of diagnosis. Proctitis was found in 57%, left-sided colitis in 12%, and substantial or total colitis in 31%. Relapse at pregnancy was demonstrated in seven patients, and remission in one. Family history of a first-degree relative with inflammatory bowel disease was documented in three patients (2%). Probable complications of ulcerative colitis were observed in 38 (26%), anemia in 13 (9%). One patient (0.7%) with rectal cancer, also had breast cancer. We suggest that the impressive increase in ulcerative colitis prevalence among Israeli and Asian-African-born, in comparison with European-American-born kibbutz members, points toward a role of environmental factors in the etiology of this disease.
Myosin heavy chain turnover during cardiac mass changes by glucocorticoids. One aim of this investigation was to determine whether the cardiac enlargement observed with glucocorticoid treatment is temporary or remains a permanent adaptation if steroid treatment is prolonged. A second aim was to study whether myosin heavy chain (MHC) synthesis rates are coordinated with the cardiac mass responses. Female rats received either a vehicle (1% aqueous carboxymethyl cellulose in saline) or hydrocortisone 21-acetate for 1, 3, 7, 11, and 15 days. Peak cardiac enlargement (10-15%) was observed after 7 days of hormone treatment in two separate series of experiments. The enlargement was maintained through 11 days of steroid injections but by 15 days had declined toward control levels. MHC synthesis measurements were performed by constant infusion of [3H]leucine. Leucine specific activities were similar among precursor pools (intracellular, extracellular, and leucyl-tRNA) and did not vary with steroid treatments. Fractional synthesis rates of ventricular MHC (%/day) did not change during the period of increase in ventricular mass but were reduced to 56-59% of controls (-11/19.5) at 7 and 11 days of treatment, when ventricular mass increases were highest. MHC breakdown (%/day) was reduced to approximately 60% (-11.5/18.7) of controls at 7 and 11 days. Changes in total protein synthesis, which was measured in isolated perfused hearts, were similar to the MHC responses and indicated that the alterations in MHC synthesis are synchronized with the hormonal effects on total protein metabolism. These results demonstrate that peak cardiac enlargement is not maintained with long-term glucocorticoid treatment.
Vitamin E supplementation and periventricular hemorrhage in the newborn. In a randomized controlled trial preterm babies received 20 mg vitamin E/kg im soon after birth (day 0) and at 24 and 48 h. The mean +/- SD plasma vitamin E level on day 0 was identical in supplemented and control groups (9.98 +/- 4.88 mumol/L) and rose progressively only in supplemented babies reaching a peak of 69.2 +/- 21.36 mumol/L at 72 h. Supplemented babies had a lower incidence of intraventricular hemorrhage (IVH) diagnosed by ultrasound (9/102, 8.8%) than the control group (37/108, 34.3%; 95% CI for difference in incidence, 15-36%). In a subsequent uncontrolled study using the same vitamin E preparation, a single dose of 20 mg/kg was given soon after birth to preterm babies. The peak mean +/- SD plasma level was 32.97 +/- 13.47 mumol/L at 48 h. The incidence of IVH (16/121, 13.2%) was lower than in historical control from the randomized trial (95% CI for difference; 10.2-31.8%) in spite of the single-dose group having more clinical risk factors for IVH. We conclude that vitamin E protects against IVH in preterm babies.
Warning: fatal reaction to the use of fibrin glue in deep hepatic wounds. Case reports. Two cases of severe hypotension following the use of fibrin glue for hemostasis in hepatic injuries are reported. A systemic reaction to bovine thrombin via large venous lacerations is suspected. A preliminary animal study supports this hypothesis. Caution is advised in the use of fibrin glue for hemostasis in deep hepatic wounds.
Long-term follow-up of the ileoanal anastomosis in children and young adults. The purpose of this study was to carry out a long-term study of the ileoanal anastomosis (IAA) in children and young adults, comparing the straight IAA to the J pouch. One hundred twenty-one young people who had undergone IAA were studied, with 114 available for long-term follow-up. One hundred one were 18 years and under. Forty-nine patients had a straight IAA and 72 had a J-pouch reservoir. There were no deaths. After surgery, three children had intraabdominal sepsis and one had pelvic sepsis, but it did not lead to excision of the IAA. The mean stool frequency in all 114 patients was 5.0 +/- 2.5 per day and 1.2 +/- 1.1 at night. The mean number of stools for the straight IAA was 6 per day and 2.1 at night. The mean number of stools for the straight IAA with balloon dilations was 5.8 per day and 1.2 at night, and for the J pouch it was 4 per day and 1 at night. Patients with both the J pouch and straight IAA had good to excellent sensation, with patients with the J pouch always able to distinguish flatus from stool in 87% of patients and almost always in 13%. Daytime continence was very good in both groups. Moderate nighttime loss of stool occurred in 10 patients, 6 with a straight IAA and 4 with a J pouch. Ninety-five percent of the 114 patients were satisfied or very satisfied, with most children with a J pouch very satisfied. The J pouch remains the procedure of choice in young people.
Epidural morphine with butorphanol for postoperative analgesia after cesarean delivery. Epidural morphine has been used more and more to provide long-lasting postoperative analgesia after cesarean delivery. However, the incidence of pruritus (20%-93%) and nausea (17%-60%) detract from the usefulness of epidural morphine. The purpose of this study was to evaluate, in 30 patients having epidural anesthesia for cesarean delivery, the analgesic efficacy and side effects when a combination of epidural morphine, a mu-receptor agonist, and butorphanol, a mu-receptor antagonist and kappa-receptor agonist, was administered. After clamping of the umbilical cord, patients received 4 mg epidural morphine with 3 mL of normal saline (group 1), 4 mg epidural morphine with 1 mg butorphanol and 2 mL of normal saline (group 2), or 4 mg epidural morphine with 3 mg butorphanol (group 3). Patients were monitored for 24 h after administration of the study medications. There were no significant differences between the groups in visual analogue pain scores, time to first analgesic request, respiratory rate, or Trieger dot test performance in the 24 h immediately after these epidural injections. There were three patients in group 1 and one patient in group 2 who experienced oxygen saturations less than 90%. (No patients in group 3 developed an oxygen saturation less than 92%.) The patients in group 3 did not require treatment for pruritus or nausea, a response significantly different (P less than 0.001 and P less than 0.05, respectively) from group 1 or group 2.