shukoor65/lab-rag-model huggingface dataset:
Desmoplastic small cell tumors of the peritoneum coexpressing mesenchymal and epithelial markers. Desmoplastic small cell tumors arising diffusely within the abdomen and lacking an apparent organ of origin are rare. Most previously reported cases occurred in children, but young adult patients also have been described. Light microscopic examination shows the tumors to be composed of nests of small cells surrounded by an abundant desmoplastic stroma. Immunohistochemical findings reveal multidirectional differentiation with coexpression of cytokeratin, milk fat globule, neuron-specific enolase, Leu-7, desmin, and vimentin. Electron microscopic examination demonstrates paranuclear condensations of intermediate filaments. The authors describe two patients who died of their disease, despite aggressive chemotherapy and surgical intervention.
Ethnic differences in mortality from ischaemic heart disease and cerebrovascular disease in England and Wales. OBJECTIVE--To examine mortality from ischaemic heart disease and cerebrovascular disease in England and Wales by country of birth of the deceased. DESIGN--Standardised mortality ratios were computed by country of birth groups for ischaemic heart disease and cerebrovascular disease for 1979-83 and 1970-2 by using the five year age-sex specific rates for England and Wales for 1979-83 as standard. SETTING--England and Wales 1970-2 and 1979-83. RESULTS--In 1979-83 mortality from ischaemic heart disease was highest in men and women born in the Indian subcontinent (standardised mortality ratio 136 and 146 respectively). Young Indian men suffered the greatest excess (313 at ages 20-29). Other groups with raised mortality included Irish, Scottish, and Polish born immigrants. Those born in the Caribbean, the old Commonwealth, west Europe, and the United States had low death rates. In England and Wales mortality from ischaemic heart disease declined by 5% in men and 1% in women between 1970-2 and 1979-83, with greatest percentage declines in immigrants born in the United States, South Africa, the old Commonwealth, the Caribbean, and France. immigrant groups with raised mortality in the earlier period showed little improvement, and mortality from ischaemic heart disease increased among Indians (6% in men and 13% in women). In 1979-83 mortality from cerebrovascular disease was highest in Caribbeans (standardised mortality ratios 176 in men and 210 in women), followed by Africans, Indians, and Irish. Rates were low in west Europeans. Mortality from stroke declined by 28% overall in this period, a rate of decline shared by most groups. Men from the Indian subcontinent showed a decline of only 3%. CONCLUSION--In the 1980s mortality from ischaemic heart disease and cerebrovascular disease differed significantly between ethnic groups in England and Wales. In general, ethnic groups that experienced lower mortality from ischaemic heart disease in the 1970s showed the greatest improvement over the following decade.
Botulinum toxin treatment of tremors. We report the results of an open trial of botulinum toxin (Botox) in the treatment of 51 patients with disabling tremors, classified as dystonic (14), essential (12), combination of dystonic and essential (22), parkinsonian (1), peripherally induced (1), and midbrain (1). The average age of the patients was 55.8 years, and duration of symptoms was 13.9 years. During a total of 160 treatment visits, an average of 242 +/- 75 units of Botox was injected per visit in cervical muscles of 42 patients with head tremor and 95 +/- 38 in forearm muscles of 10 patients with hand tremor; one patient was injected in both. The average peak effect for all patients was rated as 3.0 (0 to 4 scale). Thirty-five (67%) patients improved (peak effect greater than or equal to 1). The average latency from injection to response was 6.8 days, and the average duration of maximum improvement was 10.5 weeks. Local complications, lasting an average of 20.6 days, were noted in 17 (40%) patients injected for head tremor, consisting chiefly of dysphagia in 12 (29%), transient neck weakness in four (10%), and local pain in two (5%). Six (60%) patients with hand tremor had transient focal weakness. EMG recordings showed decreased amplitude of EMG bursts after Botox treatment. The results of this pilot study indicate that Botox injections can be used to control tremor in patients in whom other forms of therapy have failed.
Chickenpox pneumonia, its complications and management. A report of three cases, including the use of extracorporeal membrane oxygenation. We report three cases of chickenpox pneumonia in adults, all of whom required intermittent positive pressure ventilation. One patient developed a variety of complications, and another, a pregnant woman, required extracorporeal membrane oxygenation.
A prospective evaluation of the effect of tumor cell DNA content on recurrence in colorectal cancer. Tumor cell DNA (ploidy) content was measured prospectively in samples from 320 patients resected for colorectal cancer with a minimum follow-up time of 2 years. All patients were followed and those with recurrence were investigated carefully. There was no correlation between tumors with an abnormal cellular DNA content (aneuploid or tetraploid) and patient age, sex, tumor site, pathologic stage, or histologic grade. In 236 patients who underwent potentially curative operations, 75 (32%) had local and/or distant recurrence. The recurrence rate was significantly higher (test statistic, 4.3; P = 0.04) for those patients with aneuploid tumors (52 of 142, 37%) compared with those with diploid tumors (23 of 94, 24%). The subgroups of patients where ploidy exerted an effect were in patients with Stage B tumors or mobile tumors and in patients over 65 years of age. Further analysis showed that there was a twofold increase in local recurrence and a threefold increase in distant recurrence in patients with aneuploid tumors, but no excess of patients who had both local and distant recurrence. Measurement of DNA ploidy can identify a group of patients undergoing curative surgery for colorectal cancer at high risk for recurrence. In combination with clinicopathologic factors, DNA ploidy may be useful in analyzing the results of trials and in planning adjuvant therapy.
Emergency treatment of headache. Headache is a frequent presenting complaint in the emergency department. Once a diagnosis is established, and significant organic disease can be ruled out, relief of pain must be addressed. Referral for follow-up care and narcotic habituation is a recurrent problem for the emergency physician. This article discusses the differential diagnosis of headache, evaluation of the emergency room patient, and treatment of the patient with headache.
Gene deletions force nonsecretory alpha-chain disease plasma cells to produce membrane-form alpha-chain only. We studied a case of nonsecretory alpha-chain disease. The proliferating plasma cells contained a short transcript coding for a truncated membrane-form alpha 1-chain. The productive alpha-gene bore several noncontiguous deletions affecting the VHDJH and CH1 regions. Two deletions were accompanied with peculiar insertions containing duplications. The first insertion contained an acceptor splice site and was present in part in the mature transcript, thus coding for an abnormal aminoterminal peptide. Another deletion located 3' to CH3 eliminated the polyadenylation site of secreted-form alpha-mRNA. As a result, only membrane-form alpha mRNA was present in the tumoral plasma cells, thus explaining the nonsecretory phenotype of the disease. Comparison of cDNA and genomic sequences showed that the previously undescribed human alpha membrane region is encoded by a single exon, beginning with two alternate acceptor splice sites, and comprises either 65 or 71 amino acids.
Anterior sternal retraction for reoperative median sternotomy. The incidence of reoperative median sternotomy for repeat cardiac surgery is increasing. Reoperative median sternotomy is associated with a higher morbidity and mortality than first-time cardiac surgery. A portion of this morbidity and mortality may be due to direct injury to the heart and great vessels in the process of reopening the sternum. We report a new technique utilizing anterior sternal retraction that allows division of adhesions between the undersurface of the sternum and the heart and great vessels under direct vision. This technique enables the surgeon to minimize the risk of serious injury to these underlying structures during reoperative cardiac surgery.
Physical overdistension converts ventricular cardiomyocytes to acquire endocrine property and regulate ventricular atrial natriuretic peptide production. Atrial natriuretic peptide (ANP) is present in adult atria but at very low concentrations in normal adult mammalian ventricles. In the atria, the production of ANP is regulated by physical distension of the atrial wall. The same phenomenon was investigated in the ventricles of rats and men. Cardiac tissues from human ventricular aneurysm (n = 5), spontaneously hypertensive rats (n = 30), and rats that had overloaded left ventricles induced by surgery (n = 84) were studied with the methods of light microscopic immunocytochemistry, electron microscopic immunogold staining, and RNA-RNA tissue in situ hybridization. It was found that the levels of ANP gene expression, ANP immunoreactivity, and ANP-containing specific granules in the overburdened ventricles were elevated and their degrees of fluctuation were directly proportional to the force of physical distension applied to the ventricular cardiomyocytes. In rats, ANP mRNA and ANP immunoreactivity returned to the control level seven days after the ventricular overload was surgically released. The changes of ANP and its mRNA in the ventricles were related more closely to the changes of intraventricular pressure than to cardiocytic hypertrophy. In addition, ANP immunoreactivity was demonstrated in Purkinje cells and periarteriolar cardiomyocytes in the ventricles of normotensive rats. In conclusion, physical overstretch of the ventricle wall is likely to be the triggering factor affecting ventricular cardiomyocytes to acquire endocrine property, and also to regulate the production of ventricular ANP, thereby contributing to the control of the blood volume and the blood pressure.
Functional outcomes of children with sickle-cell disease affected by stroke. The nature and degree of functional recovery after stroke in children with sickle-cell disease (SCD) has not been extensively investigated. The purpose of this study was to evaluate retrospectively the functional status of 14 SCD children who had had strokes and to compare them with age-matched and gender-matched SCD children who had not had strokes. By doing so, we would be able to quantify the eventual physical and cognitive functional outcomes of survivors of stroke secondary to SCD and assess the impact of stroke on these patients. These children (five boys and nine girls) with SCD and stroke(s) were 11.6 +/- 4.3 years of age (range five to 18 years). They experienced one to three strokes at a mean age of 6.1 +/- 5.2 years (range one to 17 years). A series of tests were administered to these subjects to evaluate physical and psychosocial functions. These tests were performed at least one year after the latest stroke. This study showed that all of the SCD-stroke children were physically independent. Only a few had impairments of hand functions and mild difficulties in self-care activities. However, most of these children demonstrated intellectual deficits ranging from borderline to moderate mental retardation, reduced language functions ranging from low normal to retarded range, and problems in adjustment. Intelligence quotient of the children with SCD-stroke(s) was significantly lower than those of age-matched and gender-matched nonstroke SCD children, suggesting that stroke caused an adverse effect on the cognitive functioning of these children. The results indicate that in the SCD-stroke children psychosocial deficits outweighed physical disabilities.
Acute appendicitis. A 5-year review. A startling 31 per cent rate of perforated appendicitis in 1984 prompted a 5-year review at the Guthrie Medical Center. An increase over previous rates of 13 per cent and 0 per cent in 1964 and 1944 was confirmed in this study. Perforation accompanied 44 of 240 cases of appendicitis (18.3%); diagnostic accuracy in 295 cases undergoing operation was 81.4 per cent. Groups at risk for perforation were patients in the first decade of life (34.3% with perforations) and those over 50 years of age (48% perforated). Perforation rates were generally inversely related to accuracy. Accuracy was poorest in women in the second to fourth decade or those in the mid-portion of the menstrual cycle. When the appendix was not perforated, complications occurred in 8.7 per cent of patients while 29.5 per cent with a perforation had a complication. The mean hospital stay was prolonged by 2.5 days if the appendix was perforated. An increased awareness of the risk by both the public and physicians is essential to reduce the number of perforations.
Intrarenal pressure following pyeloplasty or percutaneous surgery. In a study of 37 patients it was shown that recording the intrapelvic pressure is a safe and reliable means of judging the best time to remove a nephrostomy tube. No post-operative complications were encountered and the procedure has the added advantage of avoiding the use of X-rays.
Oncocytic metaplasia of the pharynx. Oncocytic metaplasia of the pharynx has been infrequently described, with only two previous cases in the literature. With the advent of panendoscopy during the last decade, however, a better understanding of this histopathologic diagnosis is desirable. Thirty-three cases are reviewed, with thirty occurring in the nasopharynx. This was most commonly discovered as an unrelated finding during endoscopic evaluation of a head and neck mass or malignancy in twenty-two patients, either histologically after random biopsy or after biopsy of small but visible lesions. An additional eight cases manifested otitis media or eustachian tube dysfunction. The histology, terminology, and benign clinical nature of these lesions are discussed.
Pituitary hyperplasia and gigantism in mice caused by a cholera toxin transgene. Cyclic AMP is thought to act as an intracellular second messenger, mediating the physiological response of many cell types to extracellular signals. In the pituitary, growth hormone (GH)-producing cells (somatotrophs) proliferate and produce GH in response to hypothalamic GH-releasing factor, which binds a receptor that stimulates Gs protein activation of adenylyl cyclase. We have now determined whether somatotroph proliferation and GH production are stimulated by cAMP alone, or require concurrent, non-Gs-mediated induction of other regulatory molecules by designing a transgene to induce chronic supraphysiological concentrations of cAMP in somatotrophs. The rat GH promoter was used to express an intracellular form of cholera toxin, a non-cytotoxic and irreversible activator of Gs. Introduction of this transgene into mice caused gigantism, elevated serum GH levels, somatotroph proliferation and pituitary hyperplasia. These results support the direct triggering of these events by cAMP, and illustrate the utility of cholera toxin transgenes as a tool for physiological engineering.
Balloon dilatation of the aortic valve in the fetus: a report of two cases. Because they had irreversible damage to the left ventricular myocardium none of 12 patients with critical aortic stenosis diagnosed prenatally survived after postnatal treatment. This experience prompted three attempts at intrauterine balloon dilatation of the aortic valve in two fetuses with this condition. On each attempt the balloon catheter was successfully delivered to the left ventricle. In the first fetus the aortic valve was not crossed and the fetus died the next day. In the second fetus the balloon was correctly positioned across the aortic valve and inflated in the valve ring. After delivery, a further balloon angioplasty was performed; this relieved the stenosis but the patient died five weeks later from persisting left ventricular dysfunction related to endocardial fibroelastosis. Balloon angioplasty is feasible in fetal life but the prognosis depends on the ability of the relief of stenosis to limit, prevent, or allow regression of left ventricular damage before delivery.
New method for dealing with late-presenting spontaneous esophageal ruptures. A new technique is described for dealing with late-presenting spontaneous esophageal ruptures. This method requires only a short period of general anesthesia to drain the periesophageal abscess by a drainage tube inserted into the abscess cavity from the esophagus with the aid of a gastroscope and fluoroscopy. Gastric fluids are diverted from the esophageal rupture with a gastrostomy, and a jejunostomy is used for enteral feeding. The esophagus is retained, and closure of the fistula with resumption of normal swallowing is documented with serial sinograms.
Alfentanil for urgent caesarean section in a patient with severe mitral stenosis and pulmonary hypertension. We present the case of a parturient with severe mitral stenosis and pulmonary hypertension who received general anaesthesia using alfentanil for urgent Caesarean section. Alfentanil promoted haemodynamic stability and allowed immediate postoperative extubation. Epidural morphine provided postoperative analgesia. This combination permitted early ambulation and prevention of thromboembolism. A disadvantage of this technique, neonatal respiratory depression, was promptly reversed with a single dose of naloxone. The anaesthetic management of mitral stenosis in pregnancy is discussed and the neonatal pharmacokinetics of maternally administered alfentanil are presented.
Homozygosity for a newly identified missense mutation in a patient with very severe combined immunodeficiency due to adenosine deaminase deficiency (ADA-SCID). We have identified a previously unrecognized missense mutation in a patient with severe combined immunodeficiency due to adenosine deaminase deficiency (ADA-SCID). The mutation is a G646-to-A transition at a CG dinucleotide and predicts a glycine-to-arginine substitution at codon 216. Computer analysis of secondary structure predicts a major alteration with loss of a beta-pleated sheet in a highly conserved region of the protein. The basepair substitution also generates a new site for the restriction enzyme BstXI in exon 7 of the genomic DNA. Digestion of genomic DNA from the patient and from his parents revealed that he was homozygous for the mutation and that his mother and father were carriers. This mutation in homozygous form appears to be associated with very severe disease, since the patient had perinatal onset of clinical manifestations of SCID, the highest concentration of the toxic metabolite deoxyATP in nine patients studied, and a relatively poor immunologic response during the initial 2 years of therapy with polyethylene glycol-adenosine deaminase. Analysis of DNA from 21 additional patients with ADA-SCID and from 19 unrelated normals revealed that, while none of the normal individuals showed the abnormal restriction fragment, two of the 21 patients studied were heterozygous for the G646-to-A mutation.
Spontaneous abdominal arteriovenous fistulae: report of eight cases and review of the literature. The spontaneous rupture of an abdominal aneurysm into an adjacent major vein results in a profound and rapidly worsening haemodynamic disturbance. Survival depends on prompt diagnosis and closure of the fistula at operation. Eight cases are reported and modes of presentation, diagnostic criteria and management principles are reviewed in a detailed analysis of 148 cases in the English literature.
Hypoxaemia after nebulised salbutamol in wheezy infants: the importance of aerosol acidity. The effect of nebulised iso-osmolar, preservative free, but acidic salbutamol solution was studied in 34 acutely wheezing infants aged 1-17 months. Transcutaneous oxygen pressure (TcPO2) and oxygen saturation (SO2) fell significantly during the first five minutes after nebulisation with further deterioration at 15-20 minutes. Ten of these infants were followed up for another two hours and showed slight improvement. Even after the second hour TcPO2 had not reached baseline values. Three months later the response to salbutamol and a placebo of equal acidity (pH 3.9) was studied in 11 infants from the same group, now free of symptoms. Lung function tests were included and showed no significant changes in specific conductance and volume corrected maximum expiratory flows (Vmax at functional residual capacity/thoracic gas volume). However, hypoxaemia occurred after the acidic placebo with a significant drop of TcPO2 (mean 0.9 kPa); SO2 decreased similarly but this did not reach significance. After salbutamol there was a further significant deterioration of mean TcPO2 (1.4 kPa) and of SO2. These results show that beside a possible pharmacological effect of salbutamol the acidity of the aerosol also induces hypoxaemia in infants.
Noninvasive quantification of regional myocardial blood flow in coronary artery disease with oxygen-15-labeled carbon dioxide inhalation and positron emission tomography. BACKGROUND. Oxygen-15-labeled water is a diffusible, metabolically inert myocardial blood flow tracer with a short half-life (2 minutes) that can be used quantitatively with positron emission tomography (PET). The purpose of this study was to validate a new technique to quantify myocardial blood flow (MBF) in animals and to assess its application in patients. METHODS AND RESULTS. The technique involves the administration of 15O-labeled carbon dioxide (C15O2) and rapid dynamic scanning. Arterial and myocardial time activity curves were fitted to a single tissue compartment tracer kinetic model to estimate MBF in each myocardial region. Validation studies consisted of 52 simultaneous measurements of MBF with PET and gamma-labeled microspheres in nine closed-chest dogs over a flow range of 0.5-6.1 ml/g/min. A good correlation between the two methods was obtained (y = 0.36 + 1.0x, r = 0.91). Human studies consisted of 11 normal volunteers and eight patients with chronic stable angina and single-vessel disease, before and after intravenous dipyridamole infusion. In the normal group, MBF was homogeneous throughout the left ventricle both at rest and after administration of dipyridamole (0.88 +/- 0.08 ml/g/min and 3.52 +/- 1.12 ml/g/min, respectively; p less than or equal to 0.001). In patients, resting MBF was similar in the distribution of the normal and stenotic arteries (1.03 +/- 0.23 and 0.93 +/- 0.21 ml/g/min, respectively). After dipyridamole infusion, MBF in normally perfused areas increased to 2.86 +/- 0.83 ml/g/min, whereas in the regions supplied by stenotic arteries it increased to only 1.32 +/- 0.27 ml/g/min (p less than or equal to 0.001). CONCLUSIONS. PET with C15O2 inhalation provides an accurate noninvasive quantitative method for measuring regional myocardial blood flow in patients.