Melricflash/CW_MedAbstractsAlt huggingface dataset:
Strategy for breakpoint cluster region analysis in chronic myelocytic leukemia in a routine clinical laboratory. Despite the increasing reliance on breakpoint cluster region (bcr) determinations in diagnosis of chronic myelocytic leukemia (CML), few reports have dealt with the practical aspects of specimen analysis. In the setting of a routine molecular diagnostics laboratory, samples from 68 patients with active CML were evaluated for bcr rearrangements, with the use of a variety of enzymes and two probes. The data have been used to develop an efficient strategy for bcr screening and breakpoint determination. Screening with the universal bcr (UBCR) probe on Xba I and BgI II digests yielded bcr rearrangements in 100% of the Ph1-positive patients and three of the seven Ph1-negative patients, giving bcr analysis a sensitivity of 100%. A single-enzyme screen using the UBCR probe would have resulted in a false negative rate of 10%. The false negative rate was determined during the breakpoint site analysis from additional digests hybridized to both the 3' and UBCR probes. The false negative rate for the 3' probe was 26.5%, because of deletions or 5' rearrangements. The method of breakpoint site determination was dependent on screening results. In 78% of cases, one additional hybridization with two enzyme digests was required. During breakpoint site analysis, a rare false negative result was also demonstrated with Bam HI and Eco RI. This screening strategy has made bcr analysis competitive with cytogenetic analysis at the authors' institution; although turnaround time may be slightly longer, bcr analysis can yield information (such as detecting bcr-positive/Ph1-negative patients and determining breakpoint site) that cannot be obtained by cytogenetics. 

Category: neoplasms
Clinical classification and staging for primary malignancies of the maxillary antrum. A study of 51 patients with primary malignant maxillary sinus neoplasms was conducted. None of the patients had neck nodes and/or metastases, and each had 5-year follow-up. The tumors were staged according to the 1983 and 1988 American Joint Committee on Cancer staging systems for maxillary sinus cancers. There were 13 early stage (T1, T2) and 38 advanced (T3, T4) tumors in both systems. Cox regression analyses of survival curves showed increasingly worse prognoses for advanced tumors in both T-staging systems. Further analyses showed a significant difference in survival between T3 and T4 in the 1988, but not in the 1983 system. There were no significant differences in survival according to treatment modality or histological type of malignancy. We conclude that the 1988 system prognosticates successfully for T-stage (1 to 4) and demonstrates significant improvement in detecting T3 versus T4 differences compared to the 1983 system. The 1988 system applies equally for epidermoid cancer and other malignancies of the antrum. 

Category: neoplasms
Acute central nervous system symptoms caused by ibuprofen in connective tissue disease. We describe 2 cases of acute encephalopathy in patients with connective tissue disease caused by small doses of ibuprofen. In addition to aseptic meningitis, both patients had altered mental status and focal neurologic signs, ophthalmoplegia in one and hemiparesis in the other. The spectrum of neurologic manifestations of ibuprofen hypersensitivity is reviewed. 

Category: general pathological conditions
99TCm-HMPAO SPECT studies in traumatic intracerebral haematoma. Traumatic intracerebral haematomas are a common neurosurgical emergency. Their management, particularly the role of surgical removal, is controversial. Deterioration often occurs late, and is unpredictable. Eight patients with traumatic intracerebral haematomas were admitted to the neurosurgical unit to monitor their clinical state. All were studied within 48 hours of admission with single photon emission computerised tomography (SPECT), using the recently introduced radionuclide 99Technetiumm-Hexamethyl propylene amine oxime (99Tcm-HMPAO). At the time of the SPECT study, all the patients had been clinically stable. Three patients remained so; in the other five, the conscious level deteriorated, necessitating craniotomy and evacuation of the haematoma. In all the patients, the SPECT studies demonstrated perfusion defects that corresponded to the location of the haematoma, as demonstrated by computerised tomography (CT). However, in the five patients who subsequently deteriorated, the perfusion defects seen on the SPECT scan appeared larger than the haematoma, as seen on the CT scan. In addition, there was widespread poor retention of 99Tcm-HMPAO in the ipsilateral hemisphere. These differences were quantifiable. Interestingly, these differences were present at a time when the patients were clinically stable, before their deterioration. It is concluded that SPECT studies with 99Tcm-HMPAO are of possible use as predictors of late deterioration in the management of traumatic intracerebral haematomas. 

Category: nervous system diseases
Breast-feeding and diarrheal morbidity. This study used a unique longitudinal survey of more than 3000 mother-infant pairs observed from pregnancy through infancy. The sample is representative of infants from the Cebu region of the Philippines. The sequencing of breast-feeding and diarrheal morbidity events was carefully examined in a longitudinal analysis which allowed for the examination of age-specific effects of feeding patterns. Because the work controlled for a wide range of environmental causes of diarrhea, the results can be generalized to other populations with some confidence. The addition to the breast-milk diet of even water, teas, and other nonnutritive liquids doubled or tripled the likelihood of diarrhea. Supplementation of breast-feeding with additional nutritive foods or liquids further increased significantly the risk of diarrhea; most benefits of breast-feeding alone or in combination with nutritive foods/liquids became small during the second half of infancy. Benefits of breast-feeding were slightly greater in urban environments. 

Category: digestive system diseases
Lymphomatoid granulomatosis presenting as ulcerodestructive gastrointestinal tract lesions in patients with human immunodeficiency virus infection. A new association. We describe cases of severe odynophagia, extensive oral ulcerations, and bowel perforation in patients with human immunodeficiency virus infection that were caused by lymphomatoid granulomatosis. Such presentations in human immunodeficiency virus-infected individuals are usually ascribed to other causes and may be incorrectly treated on an empiric basis. In addition, deep tissue specimens obtained at the margin of ulcerative lesions are often necessary for definitive diagnosis. We review our limited treatment experience with zidovudine, interferon alfa, and H2 blockers in our patients. Based on the markedly increased frequency in which lymphomatoid granulomatosis is being diagnosed at our institution in the post-human immunodeficiency virus era, we postulated an association between these two entities. 

Category: neoplasms
Botulinum toxin treatment of cranial-cervical dystonia, spasmodic dysphonia, other focal dystonias and hemifacial spasm. In the past five years, 477 patients with various focal dystonias and hemifacial spasm received 3,806 injections of botulinum A toxin for relief of involuntary spasms. A definite improvement with a global rating greater than or equal to 2 on a 0-4 scale, was obtained in all 13 patients with spasmodic dysphonia, 94% of 70 patients with blepharospasm, 92% of 13 patients with hemifacial spasm, 90% of 195 patients with cervical dystonia, 77% of 22 patients with hand dystonia, 73% of 45 patients with oromandibular dystonia, and in 90% of 21 patients with other focal dystonia who had adequate follow up. While the average duration of maximum improvement lasted about 11 weeks after an injection (range seven weeks in patients with hand dystonia to 15 weeks in patients with hemifacial spasm), some patients benefited for over a year. Only 16% of the 941 treatment visits with follow up were not successful. Except for transient focal weakness, there were very few complications or systemic effects attributed to the injections. This study supports the conclusion that botulinum toxin injections are a safe and effective therapy for patients with focal dystonia and hemifacial spasm. 

Category: general pathological conditions
Failure of nine-month phenobarbital administration to reverse amygdaloid-kindled seizure susceptibility in cats. Upon completion of left amygdaloid kindling, 4 cats underwent long-term phenobarbital administration during the subsequent 5- to 9-month rest period. Plasma phenobarbital levels were maintained above 15 to 20 micrograms/ml and were restimulated following plasma phenobarbital washout. Three cats served as nonmedicated controls. All 7 cats were subjected to repeated 6-hour sleep monitoring for observation of interictal discharges, which were observed most often in the immediate postictal period. Their frequency decreased gradually throughout the experiment in both the medicated and control animals, but they never completely disappeared except from the contralateral amygdala in 1 medicated animal. Upon primary site restimulation, all of the medicated animals responded with a generalized convulsion once the afterdischarge was induced. When these animals underwent secondary-site amygdaloid kindling, 3 showed a positive transfer effect. The findings suggest that although phenobarbital is a potent anticonvulsant, it has little effect on the acquired seizure susceptibility of previously amygdaloid kindled cats. 

Category: nervous system diseases
Cytotoxic effects of high energy shock waves in different in vitro models: influence of the experimental set-up. High energy shock waves (electromagnetically generated, Siemens Lithostar) were studied for their effects in vitro on different (tumor) cell types. Cells were exposed to the shock waves as a single cell suspension or as a cell pellet on the bottom of a test tube. In both cases, a dose dependent direct cytotoxicity, established by trypan blue dye exclusion, was observed after treatment with 1000 or 2000 shock waves. Also, the antiproliferative capacity as determined by clonogenic potential (double layer soft agar) and growth rate (plastic) were affected in this way. However, comparing the results after treatment in suspension or pellet, a discrepancy was evident. The cell lines showed a different susceptibility in pellet vs. suspension. Also the differential sensitivity of the cell types varied in these two treatment models. Furthermore the outcome depended on the cell concentration; direct cytotoxicity in a cell suspension was more pronounced at higher cell concentrations, while in a pellet this was increased by decreasing the number of cells. Finally, no shock wave induced cytotoxicity could be seen after fixation of cells in gelatine or by placing the pellet on a bottom layer of gelatine. Pressure measurements revealed no adequate explanation for this phenomenon. These results indicate that in vitro effects depend on the way cells are exposed to the shock waves and can be greatly influenced by changing the conditions of the microenvironment. Therefore, precise descriptions of the experimental set-up and careful interpretations of their outcome are obligatory. 

Category: neoplasms
Symptom analysis of patients undergoing modified Pereyra bladder neck suspension for stress urinary incontinence. Pre- and postoperative findings. The results of a physician-directed follow-up of 145 women undergoing the modified Pereyra bladder neck suspension from March 1980 to February 1986 are presented. Median follow-up was 3.5 years (2.0-7.7); 70 percent of patients had follow-up between three and four years postoperatively. All patients had preoperative demonstration of stress incontinence as well as urodynamic evaluation. Fifty-one percent of patients reported no stress incontinence and 76 percent reported that their sense of urinary control was better, or much better at the time of follow-up. Improvement was seen in all grades of stress incontinence. Age, weight, parity, and history of prior anti-incontinence surgery had no significant impact on success rates. Although preoperative urgency symptoms were more common among failure (28% vs 15%), this association was not statistically significant. However, persistent or de novo urgency symptoms postoperatively were highly associated with postoperative incontinence (p less than 0.005). The onset of recurrent incontinence was experienced more than two years postoperatively in 23 percent of the incontinent group. 

Category: general pathological conditions
Twelve-year comparison of a Bjork-Shiley mechanical heart valve with porcine bioprostheses BACKGROUND. Patients undergoing heart-valve replacement may receive a mechanical prosthesis, necessitating lifelong anticoagulant treatment, or a porcine bioprosthesis, which involves no absolute need for anticoagulants. METHODS. We carried out a randomized, prospective trial to compare the durability of the Bjork-Shiley mechanical prosthesis (spherical tilting-disk model) and the incidence of valve-related complications with those variables in the Hancock and the Carpentier-Edwards porcine prostheses. The mitral valve was replaced in 261 patients, the aortic valve in 211, and both in 61; the survivors have been followed up for a mean of 12 years. RESULTS. We found a trend toward improved actuarial survival after 12 years with the Bjork-Shiley prosthesis, but this trend was not statistically significant (group with Bjork-Shiley valve vs. group with porcine valve [mean +/- SE], 51.5 +/- 3.2 vs. 44.4 +/- 3.2 percent; P = 0.08). There was no significant difference in the actuarial incidence of reoperation after 5 years, but after 12 years significantly more patients with a porcine prosthesis had undergone reoperation (8.5 +/- 2.0 vs. 37.1 +/- 4.1 percent, P less than 0.001). An analysis combining death and reoperation as end points for an actuarial assessment of survival with the original prosthesis intact confirmed that the patients with Bjork-Shiley Shiley prostheses had improved survival (48.6 +/- 3.2 vs. 30.0 +/- 3.0 percent after 12 years, P less than 0.001). Bleeding requiring hospitalization or blood transfusion was significantly more frequent in the patients with Bjork-Shiley prostheses (18.6 +/- 3.2 vs. 7.1 +/- 2.3 percent after 12 years, P less than 0.01). There was no significant difference after 12 years in the actuarial occurrence of embolism (Bjork-Shiley vs. porcine, 21.1 +/- 3.1 vs. 26.4 +/- 3.5 percent) or endocarditis (3.7 +/- 1.4 vs 4.6 +/- 1.6 percent). CONCLUSIONS. Survival with an intact valve is better among patients with the Bjork-Shiley spherical tilting-disk prosthesis than among patients with porcine bioprostheses, but use of the Bjork-Shiley valve carries an attendant increased risk of bleeding associated with the need for anticoagulant treatment. 

Category: cardiovascular diseases
The butterfly rash and the malar flush. What diseases do these signs reflect? The butterfly rash and malar flush are common facial manifestations of several disorders. Systemic lupus erythematosus may produce a transient rash before any other signs. In pellagra, symmetric keratotic areas on the face are always accompanied by lesions elsewhere on the body. Erysipelas produces brawny, fiery red facial lesions, and scarlet fever causes facial eruptions as part of a generalized eruption. Lupus vulgaris and lupus pernio produce nodules that may spread in a butterfly pattern, and seborrheic dermatitis has a predilection for the malar prominences and other areas of the face. Carcinoid syndrome often causes flushing attacks that vary in duration, and facial flushing that lasts throughout treatment may accompany chemotherapy if the patient has a hypersensitivity reaction. Deep-red rashes and/or lichenoid lesions may be caused by graft-versus-host disease in a patient undergoing bone marrow transplantation. 

Category: general pathological conditions
Clinicopathologic review of twelve children with nephropathy, Wilms tumor, and genital abnormalities (Drash syndrome). The clinicopathologic and radiologic features of 12 children with complete and incomplete forms of Drash syndrome are reported. Their common denominator was a nephropathy. Four had the full triad, consisting of nephropathy, Wilms tumor, and genital abnormalities; five had nephropathy and genital abnormalities, and three had nephropathy and Wilms tumor. Of the 11 children who had proteinuria, eight had the nephrotic syndrome. Of the 10 whose condition progressed to end-stage renal failure, seven were less than 3 years of age. The histologic features of Wilms tumor were favorable in all seven children, and the tumor was bilateral in three. Of the nine patients who had genital abnormalities, eight had 46,XY karyotype and either ambiguous genitalia (six patients) or normal female phenotype (two). One other patient had a normal 46,XX female karyotype and phenotype but had both mullerian and wolffian structures and a streak ovary. Nine patients had a distinct pelvicaliceal abnormality not previously reported as a feature of this syndrome. Other congenital abnormalities were aniridia, mental retardation, deafness, nystagmus, and cleft palate. This syndrome must be considered in any infant with unexplained nephropathy, particularly in young phenotypic female infants and in those children with ambiguous genitalia or Wilms tumor with an early presentation. 

Category: general pathological conditions
Genetics and biology of human ovarian teratomas. II. Molecular analysis of origin of nondisjunction and gene-centromere mapping of chromosome I markers. Chromosomal heteromorphisms and DNA polymorphisms have been utilized to identify the mechanisms that lead to formation of human ovarian teratomas and to construct a gene-centromere map of chromosome 1 by using those teratomas that arise by meiotic nondisjunction. Of 61 genetically informative ovarian teratomas, 21.3% arose by nondisjunction at meiosis I, and 39.3% arose by meiosis II nondisjunction. Eight polymorphic marker loci on chromosome 1p and one marker on 1q were used to estimate a gene-centromere map. The results show clear linkage of the most proximal 1p marker (NRAS) and the most proximal 1q marker (D1S61) to the centromere at a distance of 14 cM and 20 cM, respectively. Estimated gene-centromere distances suggest that, while recombination occurs normally in ovarian teratomas arising by meiosis II errors, ovarian teratomas arising by meiosis I nondisjunction have altered patterns of recombination. Furthermore, the estimated map demonstrates clear evidence of chiasma interference. Our results suggest that ovarian teratomas can provide a rapid method for mapping genes relative to the centromere. 

Category: neoplasms
Occlusion of an "accessory" distal anterior cerebral artery during treatment of anterior communicating artery aneurysms. Report of two cases. Two cases are presented in which clip occlusion of a third distal anterior cerebral artery segment occurred during treatment of anterior communicating artery aneurysms. Case histories, angiograms, operative descriptions, and postmortem findings are presented. The incidence of this anomalous vessel is reviewed. Preoperative and intraoperative vigilance in determining the presence of this anomaly prior to clip placement is emphasized. 

Category: general pathological conditions
Rett syndrome and the autistic disorders. Rett syndrome is a disorder noted to date only in females and characterized by a pervasive developmental disability following apparently normal early infancy. In addition to gait difficulties, stereotypic hand movements, and loss of communication and purposeful hand skills, autistic-like behavior is an early sign that often results in misdiagnosis. Despite these significant clinical abnormalities, neuropathologic features are modest, and no consistent laboratory abnormality or diagnostic marker has been identified. The current status of research in RS is considered within the context of autism and other disorders in which autistic features may occur, such as the fragile X syndrome. The concept of autism as neurobiologically based behavior is developed. As such, autism is regarded as an umbrella category containing an ever-expanding list of specific disorders. 

Category: nervous system diseases
Kawasaki disease: echocardiographic features in 91 cases presenting in the United Kingdom. Ninety-one patients with Kawasaki disease were examined by cross sectional echocardiography between 1980 and 1988. In the 75 patients evaluated during the acute phase of the illness (the first month), the first echocardiographic examination was carried out at a mean time of 16 days (range 5-30) and coronary arterial lesions were seen in 21 (28%). Two patients with medium sized aneurysms had myocardial infarctions, and one died. Coronary arterial lesions persisted in 17 (23%) patients, most often in younger children. The remaining 16 patients were examined from one month to four years after their acute illnesses, and this group did not have coronary arterial abnormalities. Seven patients with coronary artery lesions have reached school age and require regular echocardiographic examination and exercise electrocardiography. Selective coronary arteriography may be indicated in some patients to identify coronary artery stenosis, which the Japanese experience has shown may progress for several years after the acute illness. 

Category: general pathological conditions
Strokes in African-Americans. Hypertension is the most important risk factor for stroke, especially in African-Americans. Improved control of high blood pressure nationwide is a key factor in the recent dramatic decline in stroke frequency, most notably in African-American women. Hypertension control programs must be adequately funded and expanded. African-Americans have a disproportionately high incidence of risk factors for stroke, including hypertension. There is evidence that the cerebral vessels involved in ischemic stroke in African-Americans may differ from those of Caucasians. There is an urgent need for more research on stroke in general, risk factor relations in particular, and mechanisms in the pathogenesis of stroke in African-Americans. 

Category: nervous system diseases
Interatrial shunt flow profiles in newborn infants: a colour flow and pulsed Doppler echocardiographic study. Interatrial shunt flow profiles in 36 normal term infants were examined serially by colour flow and pulsed Doppler echocardiographic techniques from within an hour of birth to four or five days after birth. Shunt flow across the foramen ovale was detected in 33 normal infants (92%) within an hour of birth (mean 40 minutes). The occurrence of interatrial shunting decreased with age, but a shunt signal was still detected in 17 infants (47%) on the fourth or fifth day of life, by then the ductus arteriosus had already closed in all the normal infants. The direction of interatrial shunt flow was predominantly left-to-right, but in 64% there was a coexistent small right-to-left shunt in diastole within an hour of birth; by four to five days it was found in 19%. In the six patients with persistent fetal circulation the direction of the interatrial shunt flow was predominantly right-to-left with biphasic peaks in diastole and systole at the early stage of the disease, and the period of right-to-left shunt flow during each cardiac cycle was significantly longer than that in normal infants examined within 1 hour of birth. In all patients the ductus closed before the foramen ovale. At the time of ductal closure in all patients with persistent fetal circulation right-to-left shunt flow was seen during diastole and its period was still prolonged. These findings suggest that interatrial shunting, predominantly left-to-right, is common in normal newborn infants. 

Category: cardiovascular diseases