123rc/medical_text huggingface dataset:
Primary progressive aphasia. Longitudinal course, neuropsychological profile, and language features. Four patients with the clinical syndrome of primary progressive aphasia and a nonfluent aphasia profile were followed up over a period of 3 to 5 years. Extensive neuropsychological data for three patients revealed a progressive, quantitative decline of language with relative stability of memory, visuospatial skills, and reasoning. Comportment and most activities of daily living were preserved even when speech was unintelligible. Although several aphasia types may be associated with primary progressive aphasia, a nonfluent aphasia profile and phonemic paraphasic errors are most useful in differentiating it from the much more common clinical syndrome, "probable Alzheimer's disease." The clinicopathological correlates of probable Alzheimer's disease differ from those associated with primary progressive aphasia. Therefore, the clinical distinction between the two syndromes may be important for predicting the underlying pathophysiologic changes during the life of the patient.
Filler DNA is associated with spontaneous deletions in maize. We have determined the structure of five spontaneous deletions within the maize waxy (Wx) gene. Of these, four were found in spontaneous wx mutants (wx-B, wx-B1, wx-B6, wx-C4) and include exon sequences; the fifth is restricted to an intron and represents a restriction fragment length polymorphism of a nonmutant allele (Wx-W23). The deletions, which range in size from 60 to 980 base pairs (bp), cluster in a G+C-rich region of approximately 1000 bp that is capable of forming stable secondary structures. Most striking is our finding that all of the alleles have DNA insertions (filler DNA) of 1-131 bp between the deletion endpoints. For three of the five deletions, the filler DNA and sequences at the deletion termini appear to be derived from sequences near one deletion endpoint. A previously reported spontaneous deletion of the maize bronze gene (bz-R) also contains filler DNA. The association of filler DNA with maize deletion endpoints contrasts dramatically with the rarity of similar events in animal germ-line and bacterial mutations.
Fluorescent cytochemical detection of estrogen and progesterone receptors in breast fine-needle aspirates. Estrogen and progesterone receptors were studied in fine-needle aspiration biopsy specimens of 56 patients with primary, recurrent, or metastatic breast carcinoma. The ligands, 17 B-estradiol-6-carboxymethyloxine-bovine serum albumin fluorescein isothiocyanate (FITC-BSA estradiol) and hydroxyprogesterone hemisuccinate bovine serum albumin tetramethyl rhodamine isothiocyanate (TMRITC-BSA progesterone), were used in the fluorescent cytochemical method. The findings obtained from the aspirated cells with the use of the fluorescent cytochemical technique were compared with results obtained from the cell population of the same tumor after removal with the use of both the fluorescent cytochemical technique and the biochemical dextran-coated charcoal (DCC) assay. For the needle aspirates, there was 89% concordance for estrogen receptor and 86% concordance for progesterone receptor between biochemical and cytochemical results. A high degree of correlation was also demonstrated between fine-needle aspirates and imprint preparations with the use of the cytochemical technique. This study suggests that the fluorescent cytochemical technique is an effective tool in assessment of estrogen and progesterone receptor content in fine-needle aspirates of primary and metastatic breast cancer. The fluorescent cytochemical technique can be performed easily at community hospitals and is well suited for specimens of insufficient size for biochemical assay.
Clinical evaluation of plasma abnormal prothrombin (des-gamma-carboxy prothrombin) in hepatobiliary malignancies and other diseases. Des-gamma-carboxy prothrombin (DCP), a protein induced by vitamin K absence or antagonist-II (PIVKA-II) was measured in the plasma of patients with primary hepatocellular carcinoma and those with various other hepatobiliary and pancreatic diseases. DCP levels were determined by enzyme immunoassay (E-1023), using an anti-DCP monoclonal antibody. Forty-two of the 91 patients (46.2%) with hepatocellular carcinoma had abnormally elevated levels of DCP, whereas only one of the 24 patients with hepatic cirrhosis showed a slight increase. An increase was also observed in some patients with obstructive jaundice. There was no correlation between plasma levels of DCP and those of serum alpha-fetoprotein (AFP). In most patients with hepatocellular carcinoma, plasma DCP levels normalized after curative surgical resection. Plasma DCP levels were not related to the plasma concentration of vitamin K in the patients with hepatocellular carcinoma. Plasma DCP determination may be useful in the diagnosis and postoperative monitoring of the response of hepatocellular carcinoma.
Accumulation and pepsin solubility of collagens in the bowel of patients with Crohn's disease. The collagen content of resected strictured intestine, with and without fistulas, from patients with Crohn's disease has been compared with that in macroscopically normal intestine removed from the same patients and from others without inflammatory bowel disease. Collagen content per unit wet or dry weight of tissue was significantly increased in all grossly diseased tissue whether fistulated or not. Although there was a significant increase in collagen types I, III, and V in diseased tissue, the relative proportions of major collagen types extracted by limited pepsin digestion were similar for both Crohn's and non-Crohn's intestine (type I, 65 to 70 percent; type III, 25 to 30 percent; type IV, 2 to 3 percent; and type V, 2.5 to 3 percent). CNBr digestion of pepsin insoluble material showed a similar relative abundance of types I and III, indicating no major change in collagen type distribution between older (insoluble) and more newly synthesized collagen. There was no evidence of the presence of type I trimer collagen. Type VI collagen, although not quantitated, was observed in 70 percent of intestinal specimens. The proportion of total collagen solubilized by pepsin treatment was significantly greater in both grossly diseased and macroscopically normal Crohn's bowel compared with non-inflammatory bowel disease bowel. These findings suggest that there are disturbances of collagen metabolism in Crohn's intestine, which account for the stricturing process and which may predate gross pathologic changes.
Chemotherapy-related hemolytic-uremic syndrome after the treatment of head and neck cancer. A case report. A 62-year-old woman who was being treated for squamous cell carcinoma of the head and neck developed a chemotherapy-related hemolytic-uremic syndrome during the second cycle of neoadjuvant chemotherapy consisting of cisplatin, bleomycin, and methotrexate. Though the syndrome was suspected early, attempts at reversing the hematologic and renal abnormalities were unsuccessful. At postmortem examination, the characteristic microvascular lesions of the hemolytic-uremic syndrome were found in the kidneys.
Acute phase reactants and risk of bacterial meningitis among febrile infants and children. STUDY OBJECTIVE: To test the hypothesis that quantitation of either C-reactive protein (CRP) or the total peripheral WBC count can improve clinical detection of underlying bacterial meningitis among young febrile children. DESIGN: Cross-sectional survey of selected symptoms of central nervous system infection, signs of meningeal irritation and/or elevated intracranial pressure, levels of CRP in serum, and total peripheral WBC counts among unselected pediatric patients undergoing lumbar punctures for evaluation of acute febrile illnesses. SETTING: Emergency department and acute care "walk-in" clinic of an urban, university-affiliated general hospital. PARTICIPANTS: 160 previously well, acutely febrile infants and children (median age, 6 months). RESULTS: The prevalence of bacterial meningitis was 6%. Sensitivity of symptoms was 1.00 and specificity was 0.17. Sensitivity of signs was 0.70 and specificity was 0.81. Of the acute phase reactants, sensitivity of a CRP level of more than 1.0 mg/dL was 0.80, while that of a total peripheral WBC count of more than 15,000/mm3 was 0.40. The presence of signs and/or a CRP level of more than 1.0 mg/dL correctly identified all children with bacterial meningitis (sensitivity, 1.00). The absence of signs and a CRP level of 1.0 mg/dL or less correctly identified 71 of 150 children without bacterial meningitis (specificity, 0.47). Of 125 children without meningeal signs, the combination of symptoms and a CRP level of more than 1.0 mg/dL correctly identified all three children with bacterial meningitis (sensitivity, 1.00). The absence of these symptoms and/or a CRP level of 1.0 mg/dL or less correctly identified 80 of 122 children without bacterial meningitis (specificity, 0.66). CONCLUSION: Quantitation of CRP but not the total peripheral WBC count can increase the sensitivity of physical examination findings and the specificity of symptoms for the diagnosis of bacterial meningitis. Measurement of CRP in serum is useful as an adjunct to history and physical examination for the detection of acute bacterial meningitis in the acutely febrile child.
Striatonigral degeneration. A clinicopathological study. The clinical and pathological features of 10 cases of striatonigral degeneration are described: 5 were misdiagnosed in life as Parkinson's disease. Retrospectively, helpful early pointers to the diagnosis in these cases included unexplained falls, autonomic dysfunction, absence of rest tremor and failure to respond to L-dopa, but these were not always present. The pathological diagnosis could not be excluded on macroscopic examination of the striatum. Relative preservation of the putamen occurred in the 4 patients who benefited from L-dopa. The caudate nucleus was involved in all cases and there was no sparing of the large striatal neurons. In mild cases, involvement of the putamen was confined to its posterior two-thirds, dorsolaterally. With increasing severity this extended in a dorsal to ventral and posterior to anterior direction. Seven of the cases had evidence of olivopontocerebellar damage, but only 2 of these had clinical evidence of cerebellar disease. Correlation was found between the neuronal counts in caudate:putamen, striatum:nigra compacta, globus pallidus:nigra compacta, nigra compacta:locus coeruleus. The most severely involved part of the substantia nigra pars compacta was the ventrolateral zone, which projects to the dorsal putamen, the earliest site of striatal disease.
Primary osteosarcoma of the lung. Report of two cases and review of the literature. Two cases of primary osteosarcoma of the lung are presented. In one case, the radiologic, clinical, and cytologic findings led to a preoperative diagnosis of undifferentiated carcinoma of the lung. In the second case, a lung nodule was discovered during postchemotherapy follow-up in a patient with lymphoma. Fine needle aspiration in the second case showed lymphoma, and further chemotherapy was instituted; however, persistent growth of the nodule prompted a resection. Microscopic examination of the resected tumors in both cases revealed histologic features of high-grade osteosarcoma. Flow cytometric analyses of the primary tumors showed abnormal hyperdiploid deoxyribonucleic acid populations in accordance with those seen in high-grade malignant neoplasms. Immunohistochemical studies supported a mesenchymal origin for these tumors. These tumors shared clinical features with other reported cases of primary osteosarcoma of the lung such as large size at diagnosis, occurrence in older individuals, and aggressive behavior.
Marked elevations of serum alkaline phosphatase in patients with AIDS. We have observed many patients with AIDS who have unexplained marked elevations in serum alkaline phosphatase. To determine the frequency of alkaline phosphatase elevations in patients with AIDS, and to identify diagnoses, medications, and demographic factors associated with such elevations, we conducted a retrospective study of the first 90 consecutive AIDS cases in hospitals affiliated with the University of Colorado Health Sciences Center in Denver, Colorado. We found elevations of alkaline phosphatase in excess of 1,000 IU/L in 17% of consecutive patients with AIDS. This level of elevation was less frequent in patients with Kaposi's sarcoma but there was otherwise no significant association with diagnoses or medications, or transmission categories for AIDS. The majority of the patients with elevations to this level did not have documented opportunistic infections or biliary tract dilatation previously described in the "cholangitis" syndrome in AIDS patients. Other explanations for these elevations for this common laboratory finding may exist.
Replacement of an aortic valve cusp after neonatal endocarditis. Septic arthritis developed in a neonate after an infection of her hand. Despite medical and surgical treatment endocarditis of her aortic valve developed and the resultant regurgitation required emergency surgery. At operation a new valve cusp was fashioned from preserved calf pericardium. Nine years later she was well and had full exercise tolerance with minimal aortic regurgitation.
Arterial aneurysms in children: clinicopathologic classification. Thirty-one arterial macroaneurysms in 23 pediatric-aged patients (16 boys and 7 girls) were treated at the University of Michigan. The average age at time of diagnosis was 10.2 years (range 6 months to 18 years). Vessels involved the aorta (4), as well as hepatic (1), splenic (2), gastroepiploic (1), renal (12), iliac (1), superficial femoral (4), popliteal (1), brachial (1), radial (2), and ulnar (2) arteries. Twelve children exhibited overt clinical manifestations including presence of a mass (7), local pain (3), hematemesis (1), and painless obstructive jaundice (1). Eleven children had asymptomatic lesions. Aneurysm existence was confirmed by arteriography or operation. All but one child underwent surgical therapy, with 20 long-term survivors (mean follow-up 3.5 years). One operative death occurred and one death occurred 6 years after surgery. This experience and a review of previously reported cases served as a basis for categorization of childhood aneurysmal disease as true aneurysms associated with (I) arterial infection, (II) giant-cell aortoarteritis, (III) autoimmune connective tissue disease, (IV) Kawasaki's disease, (V) Ehlers-Danlos syndrome or Marfan's syndrome, (VI) other forms of noninflammatory medial degeneration, (VII) arterial dysplasias, (VIII) congenital-idiopathic factors, as well as (IX) false aneurysms associated with extravascular events causing vessel wall injury or disruption. Knowledge of the varied clinicopathologic characteristics of arterial aneurysms in children is important in treating these patients.
Adverse mortality experience of a southwestern American Indian community: overall death rates and underlying causes of death in Pima Indians. As part of an ongoing epidemiologic study, the death rate and causes of death during 1975 through 1984 were determined in Pima Indians who resided in the Gila River Indian Community (GRIC) in 1965 and later. Death certificates were available for 677 of the 681 deaths. In 78% of the deaths, the underlying cause recorded on the death certificate agreed with the cause determined after review of all available relevant records. The age- and sex-adjusted average annual death rate for the GRIC population (1639/100,000) was 1.9 times (95% CI 1.7-2.0) the 1980 rate for the U.S. all races (878/100,000). In Pima males, whose death rate was substantially higher than that of Pima females, the age-adjusted death rate was 2.3 times that in U.S. males, all races. Moreover among males 25-34 years of age, the Pima death rate was 6.6 times that for the U.S. all races. Diseases of the heart and malignant neoplasms caused 59% of U.S. deaths in 1980, but only 19% of GRIC deaths. By contrast, the age- and sex-adjusted mortality rate in the GRIC Pima was 5.9 times the rate of the U.S. all races for accidents, 6.5 times for cirrhosis, 7.4 times for homicide, 4.3 times for suicide, and 11.9 times for diabetes. Tuberculosis and coccidioidomycosis were important causes of death in the Pima, for whom infectious diseases was the tenth leading cause of death. The findings indicate that programs to improve the adverse mortality experience of the GRIC population should emphasize factors related to fatal accidents, alcoholic cirrhosis, homicide, suicide, diabetes mellitus, and infectious diseases. Young Pimas, especially the males, should be the primary focus of such preventive efforts. These findings and recommendations probably apply to many Native American populations.
Mammographic measurements before and after augmentation mammaplasty. Thirty-five augmented women underwent mammography using both the standard implant-compression technique and, when possible, the implant-displacement technique; all had preaugmentation film-screen mammography available for evaluation. The area of mammographically visualized breast tissue before and after augmentation mammaplasty was measured using a transparent grid. Patients with subglandular implants had a mean decrease of 49 percent of measurable tissue area with compression mammography and a 39 percent decrease with displacement mammography. Patients with submuscular implants had a 28 percent decrease in measurable tissue area with compression mammography and a 9 percent decrease with displacement mammography. Anterior breast tissue was seen better with displacement mammography; posterior breast tissue, with compression mammography. Most patients had some degree of parenchymal scarring and lower image quality after augmentation. State-of-the-art mammography was not possible in most patients augmented with silicone-gel-filled implants.
Features distinguishing secondary achalasia from primary achalasia. Eighteen patients with cancer-induced or secondary achalasia (SA) were compared to 421 patients with idiopathic or primary achalasia (PA). The aim of the study was to detect any differences in clinical presentation between the two groups. Mean age of patients with SA was 57.1 (range 15-78) and 47.1 (range 1-90) in patients with PA (p = 0.02). Three patients with SA were 15, 24, and 36 yr old, respectively. Symptom frequency was comparable in SA versus PA. Mean duration of symptoms in SA was 4.5 months, with 15 of the 18 patients experiencing symptoms for six months or less. Weight loss occurred in 88.2% of patients with SA and 57.3% of patients with PA (p less than 0.05). Cancer was at the gastroesophageal junction in 16 patients, duodenum in one, and breast in one. Endoscopy showed tumor in 12 (67%). The esophagram was suspicious for tumor in only 25%. We conclude that patients with SA are older, more likely to lose weight, and have a short duration of symptoms. However, SA may occur in younger patients, and endoscopy with biopsy is necessary in any newly diagnosed case of achalasia.
An objective method for the assessment of psychosocial problems in adolescents with epilepsy. Psychosocial problems in adolescents with epilepsy have been of concern for many years, but have been difficult to assess. This article presents the multicenter development of the Adolescent Psychosocial Seizure Inventory (APSI), an empirically based self-report test patterned after the Washington Psychosocial Seizure Inventory, which is used to evaluate psychosocial problems in adults. After pilot work, 120 adolescents with epilepsy from five centers in North America took the APSI and were interviewed by professionals with respect to adequacy of adjustment in eight psychosocial areas. At least one parent or guardian was also interviewed. Interrater reliability of professional ratings in each area was established. Using an item-by-item, empirically based technique, eight psychosocial scales were developed as well as three validity scales. Reliability of the scales was established by both internal consistency and test-retest procedures. Results for each adolescent are presented in profile form. These results give a visual display of the types and extent of problems that likely would be identified in a detailed professional assessment. It is anticipated that the APSI will be of value in a variety of treatment and research contexts.
Balloon dilatation of the mitral valve by a single bifoil (2 x 19 mm) or trefoil (3 x 15 mm) catheter. The efficacy of balloon dilatation of the mitral valve by a bifoil (2 x 19 mm) or trefoil (3 x 15 mm) catheter (single catheter technique) was assessed in 53 patients (mean age 28) with mitral stenosis, most of whom were women. The procedure was unsuccessful in three patients. After balloon dilatation the left atrial pressure decreased from 22 mm Hg to 13 mm Hg and the mitral valve gradient from 12 mm Hg to 4 mm Hg. The mitral valve area increased from 0.7 cm2 to 2.1 cm2. Exercise time on the standard Bruce protocol increased from 3.9 minutes to 7.2 minutes. In 22 (44%) patients mitral regurgitation developed or the grade of regurgitation increased. Left to right shunts with pulmonary to systemic flow ratios greater than 1:5 were detected in four patients. Transient cerebrovascular episodes developed in two patients. One patient died after emergency valve replacement for severe mitral regurgitation. Balloon dilatation of the mitral valve by the single catheter technique with the bifoil or trefoil catheters is an effective treatment for patients with mitral stenosis. Mild mitral regurgitation is a frequent complication of the procedure.
Cauda equina anatomy. I: Intrathecal nerve root organization. The three-dimensional organization of the human cauda equina has not been described previously. This is partly due to the difficulties of dissecting individual, unfixed nerve roots. By the use of a newly developed in situ fixation and embedding technique on 15 fresh human cadavers, the cross-sectional anatomy of the cauda equina was defined from L2-L3 to L5-S1. A highly consistent cross-sectional pattern was observed in all specimens. The lower sacral (S2-S5) and coccygeal roots were located in the dorsal aspect of the thecal sac, whereas the lumbar and first sacral roots exhibited an oblique, layered pattern as they ascended. The motor bundle was situated anteromedial to its respective sensory bundle within each layer, Invaginations of arachnoid held the nerve roots in a fixed relationship to one another. This previously undescribed three-dimensional anatomy within the thecal sac may aid in the understanding and treatment of trauma, neurocompressive syndromes, and tumors of the cauda equina.
Differences between multisite postmortem ethanol concentrations as related to agonal events. In a study of postmortem ethanol concentrations, blood was withdrawn from the right atrium, ascending aorta, and inferior vena cava. These samples, vitreous humor, and gastric fluid were analyzed in 307 autopsies, where a minimum blood ethanol concentration of 0.05% weight/volume (w/v) was present. Premortem, agonal, and postmortem events were reviewed in an attempt to account for differences in blood ethanol concentrations between sites. The agonal aspiration of vomitus having at least 0.80% w/v ethanol appears to be associated with an increase in aortic ethanol concentrations. We conclude that valid interpretation of postmortem ethanol concentrations must take into consideration the possible entry of ethanol into the pulmonary venous circulation via the respiratory system.