Melricflash/CW_MedAbstractsAlt huggingface dataset:
Trichothiodystrophy with chronic neutropenia and mild mental retardation. Trichothiodystrophy is a feature of several diseases that consist of characteristic hair shaft abnormalities and a wide spectrum of other developmental defects. Detection of sulfur-deficient hairs identifies this disorder and separates it from other similar ectodermal dysplasias with normal sulfur content. Detection of low sulfur hair syndrome is also important for genetic counseling, because the disease appears to be an autosomal recessive trait. We report a patient with chronic neutropenia, mild mental retardation, and low sulfur content in hair. Our case expands the spectrum of disorders associated with trichothiodystrophy. 

Category: nervous system diseases
Improved cannulation method for extracorporeal membrane oxygenation. Extracorporeal membrane oxygenation has been shown to be useful for patients in reversible cardiogenic shock. Effective arterial cannulation techniques for infants have been developed that are simple to use and require minimal subsequent vascular repair or reconstruction after removal. Groin cannulation in adults frequently requires bidirectional arterial cannulation to ensure adequate distal perfusion as well as frequent complex arterial repairs after discontinuation. We describe a simple arterial cannulation technique using a single right-angle, high-flow arterial cannula. With this technique adequate bidirectional arterial perfusion is maintained with a single arterial cannula while the need for vascular repairs or reconstruction is minimized. 

Category: cardiovascular diseases
A comparison between single and double dose intravenous Timentin for the prophylaxis of wound infection in elective colorectal surgery. A prospective, randomized, single-blind, controlled clinical trial was undertaken to determine whether two doses of systemic Timentin provided superior prophylaxis against postoperative sepsis in elective colorectal surgery compared with a single dose of the same antibiotic. Timentin, a combination of ticarcillin and clavulanic acid was administered intravenously (3.1 g) at the commencement of operation to all patients, and this was repeated after 2 hours in those patients randomized to receive a second dose. The wound infection rate was 11 percent in the 143 patients completing follow-up and receiving a single dose, and 13 percent in the 128 patients receiving two doses of Timentin (P greater than 0.05). The rates of postoperative septicemia 3 vs. 4 percent and intra-abdominal abscess 5 vs. 8 percent were similar. Multivariate analysis of the factors likely to affect postoperative would infection rate demonstrated an association with the type of hospital, public or private, wound infection rate 16 and 6 percent, respectively (P less than 0.01), and the surgeon group defined by the number of patients contributed greater than 25 or less than 25, wound infection rate 6 and 18 percent, respectively (P less than 0.05). We concluded that a single dose of intravenous Timentin was as effective as two doses for prophylaxis against surgical infection and that the surgeon group and the hospital in which the operation took place were statistically significant predictors of postoperative wound infection. 

Category: general pathological conditions
Coronary angioplasty in patients with severe left ventricular dysfunction. The applications for coronary angioplasty have greatly expanded and the procedure is now increasingly used in complex and potentially high risk conditions. This report describes the short- and long-term effects of coronary angioplasty in 61 patients with severely depressed left ventricular function (ejection fraction less than or equal to 35%) with unstable or refractory anginal symptoms, or both, in whom revascularization was necessary despite increased risk. In a retrospective analysis of 1,260 patients undergoing angioplasty between January 1985 through December 1987, 61 had an ejection fraction less than or equal to 35%. The common clinical presentation was unstable angina (70%) with or without recent myocardial infarction. Mean left ventricular ejection fraction was 27 +/- 6%. Forty-five patients (74%) had multivessel disease. Clinical success after angioplasty was achieved in 55 patients (90%). Major complications (death, infarction and emergency bypass surgery) occurred in five patients (8.2%), with death in two (3.2%). During long-term (mean 21 +/- 11 months) follow-up study of the 55 patients with successful angioplasty, 13 (23%) died, including 3 of noncardiac causes, and 11 (20%) had clinically symptomatic recurrence. Continued clinical success was present in 39 patients (71%), of whom 28 (51%) were event-free patients and 11 (20%) had clinical recurrence; a successful second angioplasty procedure was performed in 9 because of restenosis. Thus, in patients with depressed left ventricular function, coronary angioplasty can be performed with a short-term success rate comparable to that of routine angioplasty or surgical procedures. However, acute complications are more frequent and the late mortality rate is higher than in patients with less depressed function. 

Category: cardiovascular diseases
The association of carotid artery stenosis with carotid sinus hypersensitivity. Transitory cerebral ischaemic attack provoked by carotid sinus reflex The association of internal carotid stenosis with the carotid sinus syndrome represents a special clinical entity. Transitory cerebral ischaemic attack (TIA) will usually be manifested by activation of carotid sinus reflex. Eighteen patients were observed suffering from both carotid sinus hypersensitivity and TIA. The patients had had TIA's for many years. Unilateral internal carotid stenosis was detected in 15 cases, while 3 patients had bilateral carotid stenosis. On carotid sinus stimulation, syncope appeared and a TIA could be provoked. The mean duration of the syncopic attack was 5500 ms and was based on sinus arrest in 14 cases and on third degree AV block in 4 cases. In all patients carotid artery disobliteration was performed; in 14 patients pacemaker implantation was necessary, while 4 patients could be treated by atropine medication. The development of a TIA could be abolished in every patient and 14 patients remained totally symptom free. 

Category: nervous system diseases
Transient neuromuscular impairment resulting from prolonged inhalation of halothane and enflurane. Inhalation anesthesia first with halothane followed by enflurane relieved a patient with status asthmaticus who was refractory to conventional therapy including mechanical ventilation. After 13 days of anesthesia while on mechanical ventilation and employing nondepolarizing muscle relaxants, significant neuromuscular impairment, manifested by tetraplegia and sensory disturbance, developed. Anesthesia was discontinued on day 14, and the patient was weaned from mechanical ventilation on day 16. Over the next two months, the neuromuscular impairment markedly improved. Halothane was associated with cardiac arrhythmias and hepatitis necessitating replacement by enflurane. Enflurane appeared to be as effective a treatment for refractory asthma as halothane. The most probable cause of the neuromuscular impairment in our patient was the long-term use of inhalation anesthetics or nondepolarizing muscle relaxants. 

Category: nervous system diseases
Selective evaluation and management of coronary artery disease in patients undergoing repair of abdominal aortic aneurysms. A 16-year experience. Reduction of cardiac mortality associated with abdominal aortic aneurysm (AAA) repair remains an important goal. Five hundred consecutive urgent or elective operations for infrarenal nonruptured AAA were reviewed. Patients were divided into three groups based on preoperative cardiac status: group I (n = 260, 52%), no clinical or electrocardiographic (ECG) evidence of coronary artery disease (CAD); group II (n = 212, 42.2%), clinical or ECG evidence of CAD considered stable after further evaluation with studies such as dipyridamole-thallium scanning, echocardiography, or coronary arteriography; group III (n = 28, 5.6%), clinical or ECG evidence of CAD considered unstable after further evaluation. Group I had no further cardiac evaluation and groups I and II underwent AAA repair without invasive treatment of CAD. Group III underwent repair of cardiac disease before (n = 21) or coincident with (n = 7) AAA repair. In all instances, perioperative fluid volume management was based on left ventricular performance curves constructed before operation. The 30-day operative mortality rate for AAA repair in all 500 patients was 1.6% (n = 8). There was one (0.4%) cardiac-related operative death in group I, which was significantly less than the five (2.4%) in group II (p less than 0.02). Total mortality for the two groups were also significantly different, with one group I death (0.4%) and seven group II deaths (3.3%), (p less than 0.02). These data support the conclusions that (1) the leading cause of perioperative mortality in AAA repair is myocardial infarction, (2) correction of severe or unstable CAD before or coincident with AAA repair is effective in preventing operative mortality, (3) patients with known CAD should be investigated more thoroughly to identify those likely to develop perioperative myocardial ischemia so that their CAD can be corrected before AAA repair, and (4) patients with no clinical or ECG evidence of CAD rarely die of perioperative myocardial infarction, and thus selective evaluation of CAD based on clinical grounds in AAA patients is justified. 

Category: cardiovascular diseases
Control of interleukin-1 beta expression by protein kinase C and cyclic adenosine monophosphate in myeloid leukemia cells. We have examined the signal transduction pathways leading to the expression of the interleukin-1 beta (IL-1 beta) gene in human myeloid leukemia cells lines. Two cell lines representing different stages of differentiation were used (HL-60, promyelocytic, and THP-1, mature monocytic). In accordance with previous studies, it was observed that a protein kinase C (PKC) activator, phorbol myristate acetate (PMA), was a sufficient stimulus for induction of the IL-1 beta messenger RNA (mRNA) expression and IL-1 beta protein production in both of these cell lines. A structural analog of cyclic adenosine monophosphate (dbcAMP) or agents elevating the endogenous cAMP levels (prostaglandin E2, forskolin) were not alone able to induce IL-1 beta expression, but they strongly enhanced the PMA-induced IL-1 beta production and IL-1 beta mRNA accumulation. Nuclear run off analysis showed that this elevation in IL-1 beta mRNA levels was due to an increased rate of transcription. If dbcAMP was added 6 hours before PMA to the cultures, no enhancement in the IL-1 beta production was seen, implying that for this enhancing effect both of these signals must be present simultaneously. PKC inhibitor, H7, also blocked effectively the PMA plus dbcAMP induced IL-1 beta production, while the protein kinase A (PKA) inhibitor, HA1004, had no effect, suggesting that PKA activation is not involved in the mechanism of action of cAMP in this case. Collectively, the present findings show that cAMP-dependent signals can have a positive regulatory effect on the PKC-dependent activation of the IL-1 beta gene in cells derived from different stages of myeloid differentiation. 

Category: neoplasms
Fetal death due to nonlethal maternal carbon monoxide poisoning. Fetal death due to acute carbon monoxide poisoning is rarely reported in the medical literature. Of the eight cases found in literature review, only one documented the fetal carboxyhemoglobin concentration. This paper reports a fetal death due to accidental nonlethal maternal carbon monoxide intoxication in which both maternal and fetal carboxyhemoglobin concentrations were obtained. The corrected carboxyhemoglobin concentration was 61% at the time of death in utero, while the maternal carboxyhemoglobin was measured at 7% after one hour of supplemental oxygen. The authors review the mechanisms of fetal death and emphasize the different carbon monoxide kinetics in the fetal circulation. 

Category: general pathological conditions
Nonpigmented congenital iris stromal cyst. A nonpigmented iris stromal cyst was observed in the right eye of a 6-week-old girl. There was no past history of trauma or use of topical miotics, and the remainder of the ocular examination was normal. The pathophysiology and management of this rare developmental anomaly are controversial. 

Category: neoplasms
The current spectrum of peptic ulcer disease in the older age groups. Not only has there been a relative increase in the prevalence of peptic ulcer disease (PUD) among America's older age groups, but the characteristics of PUD in these patients differ significantly from those of the general population. Seventy-two consecutive patients 60 years of age or older who underwent operation for PUD between 1984 and 1989 were studied. The unusual features in these patients were 1) 92 per cent required emergency operation, 2) 57 per cent with perforated PUD were female, 3) 85 per cent had duodenal pathology, 4) 28 per cent were currently taking nonsteroidal anti-inflammatory agents, and 5) over one half of all patients had serious postoperative complications. The increasing incidence and associated serious complications of PUD in the elderly population present new challenges to physicians in the diagnosis and treatment of this disease. 

Category: general pathological conditions
Urticaria: current therapy. Although the ideal treatment for urticaria is identification and removal of its cause, no underlying cause can be discerned in the majority of instances. The chief clinical problem is the treatment of chronic idiopathic urticaria. H1-receptor antagonists are the major class of therapeutic agents used in the management of chronic idiopathic urticaria. The H1 antagonists have been divided into subgroups based on their chemical structure. The second-generation H1 antagonists now available are particularly advantageous for individuals who must remain alert while working. Terbutaline, a beta-adrenergic agonist, is of occasional benefit as an adjunct therapy in combination with an H1 antagonist. The oral administration of disodium cromoglycate is ineffective in patients with chronic idiopathic urticaria, although a few individuals with urticaria caused by food allergy may respond to this drug. It is best to avoid repeated injections of epinephrine and the systemic administration of corticosteroids. Urticaria has a capricious course: it may respond to the administration of placebos or it may resolve spontaneously. About 50% of the patients with urticaria are free of lesions within 1 year, but 20% continue to have episodes for more than 20 years. 

Category: cardiovascular diseases
Malignant melanoma presenting as nasal obstruction. Mucosal melanomas arising in the nasal cavity are rare tumors comprising less than 1 percent of all melanomas. Often, the common clinical symptom is nasal obstruction. Grossly, they may or may not be pigmented and frequently attain large sizes. Histologic diagnosis of these tumors may be difficult, requiring immunohistochemical or electron microscopic confirmation. Aggressive surgical management is the treatment of choice in clinical stage I disease. Subsequent surveillance for recurrence is mandatory. Markers such as 5-S-cysteinyldopa may prove useful in staging, prognosticating, and postoperative surveillance for early recurrence, but their exact role has yet to be delineated. Ultimate prognosis is poor. 

Category: neoplasms
Initial evaluation of heart murmurs: are laboratory tests necessary? Heart murmurs, most of them innocent, are the most common reason for referrals to a pediatric cardiologist. In the evaluation of murmurs, the electrocardiogram and echocardiogram are often included. The purpose of this study was to determine the utility of these examinations in the initial assessment of heart murmurs in children and adolescents. In a prospective series of 161 patients, the clinical diagnosis of heart murmurs by a pediatric cardiologist was compared with that obtained after electrocardiogram and echocardiogram (two-dimensional, M-mode, Doppler, and color-Doppler). On the basis of the clinical diagnosis the patients were classified as having "innocent murmur," "pathologic murmur," or "possible pathologic murmur." A total of 161 patients (51% males), aged 1 month to 17 years (median 3.2 years), were studied. After electrocardiogram, no diagnosis was changed. After echocardiogram, the clinical diagnosis of innocent murmur in 109 patients changed in 2 to pathologic (small ventricular septal defect 1, small atrial septal defect 1); pathologic murmur in 46 changed to innocent in 3 and possible pathologic in 2; and possible pathologic in 6 changed to innocent in 3 and to pathologic in 2. The clinical examination by an experienced pediatric cardiologist is an accurate means of assessing newly referred patients with murmurs. The clinical examination had a sensitivity of 96%, specificity of 95%, positive predictive value of 88%, and negative predictive value of 98%. The electrocardiogram, unlikely to disclose any unsuspected heart disease, may assist in reaching the lesion-specific diagnosis when there is underlying pathology. Echocardiography, although diagnostic when heart disease is suspected, is unnecessary in pediatric patients with clinically diagnosed innocent heart murmurs. 

Category: cardiovascular diseases
Noninvasive estimation of left atrial pressure in patients with congestive heart failure and mitral regurgitation by Doppler echocardiography. A completely noninvasive method for estimating left atrial pressure in patients with congestive heart failure and mitral regurgitation has been devised with the use of continuous-wave Doppler echocardiography and brachial sphygmomanometry. Of 46 patients studied with mitral regurgitation, 35 (76%) had jets with distinct Doppler spectral envelopes recorded. The peak ventriculoatrial gradient was obtained by measuring peak mitral regurgitant velocity in systole and using the modified Bernoulli equation. This gradient was then subtracted from peak brachial systolic blood pressure, an estimate of left ventricular systolic pressure, to yield left atrial pressure (left atrial pressure = systolic blood pressure - mitral regurgitant pressure gradient). Noninvasive estimates of left atrial pressure from 35 patients were plotted against simultaneous recordings of mean pulmonary capillary wedge pressure resulting in the correlation y = 0.88x + 3.3, r = 0.88, standard error of estimate = +/- 4 mm Hg (p less than 0.001). Therefore, continuous-wave Doppler echocardiography and sphygmomanometry may be used in selected patients with congestive heart failure and mitral regurgitation for noninvasive estimation of left atrial pressure. 

Category: cardiovascular diseases
End-stage renal disease--is infrainguinal limb revascularization justified? Reports of reconstructive surgery for peripheral vascular disease have been relatively uncommon in patients with end-stage renal disease. Between 1980 and 1989, 39 patients with end-stage renal disease underwent revascularization of 56 limbs. Fifty-two primary infrainguinal and four secondary infrainguinal bypass grafts were performed. In addition, nine thrombectomies were performed. At the time of surgery 37 patients were on dialysis; three had functioning kidney transplants. The indications for revascularization were gangrene, rest pain, or ulceration in all except three limbs with disabling claudication. Reversed, nonreversed, or in situ vein was used in 25 of the 52 primary infrainguinal revascularizations performed. Polytetrafluoroethylene was used in 25. Two procedures used a combination of polytetrafluoroethylene and vein. The primary patencies for all infrainguinal procedures at 1 and 2 years were 77% and 68%, respectively. Four perioperative deaths occurred in the infrainguinal group (7.7%). An additional death occurred after thrombectomy for late graft closure. Three deaths were a result of myocardial infarction. One patient on peritoneal dialysis developed uncontrolled sepsis. At 3 years 39% of patients were alive, and 84% of the limbs were salvaged. Among the cases studied no group was identified that represented unacceptable operative risk. Results compared well with reported patencies for patients subjected to infrainguinal revascularization procedures. Limb revascularization in patients with end-stage renal disease may be performed by use of similar criteria to those used for other patients with peripheral vascular disease. 

Category: cardiovascular diseases
Persistent mullerian duct syndrome. Persistent mullerian syndrome is rare. A case of phenotypically normal male with persistent mullerian structures that consisted of a bicornuate uterus, fallopian tubes, and upper third of the vagina is reported. These unusual structures were found in association with bilateral cryptorchidism and a right inguinal hernia, and were diagnosed while repairing the hernia in the neonatal period. The uterus and fallopian tubes were removed via laparotomy when the child was eighteen months old; at the same time a bilateral orchiopexy was performed. To avoid damage to the vas deferens, which lay in closely to the mullerian structures and could not be separated from the vaginal wall, a small segment of the upper third of the vagina was retained. The testicles, although normal on pathologic examination, have shown poor response to hormonal stimulation with human chorionic gonadotropin. Long-term follow-up for these patients is necessary because they have an increased risk of testicular tumors developing. 

Category: digestive system diseases
Psychosocial adjustment in women with breast cancer. There is a plethora of studies investigating psychosocial adjustment in women with breast cancer, its correlates, clinical course, and prognosis. These studies have been conducted with varying degrees of methodologic rigor. An assessment has been made of the quality of this existing evidence to identify from the best evidence the factors which predict the adjustment status of women with breast cancer. Studies have been reviewed, using methodologic standards for the critical appraisal of studies on prognosis, developed by Sackett and colleagues in the Department of Clinical Epidemiology and Biostatistics at McMaster University (Hamilton, Ontario, Canada). Few of the studies investigating psychosocial adjustment of women with breast cancer meet all of the criteria for reviewing studies of clinical course and prognosis. This review focuses the direction and methodologic rigor required in future investigations. In particular, studies are needed that employ prospective designs and that deliberately measure or control for the extraneous prognostic variables that may affect adjustment. Future investigations need to incorporate adequate precision in measurement so that measures of the psychosocial variables are objective, reliable, and valid. 

Category: neoplasms