Melricflash/CW_MedAbstractsAlt huggingface dataset:
Parental age in sporadic hereditary retinoblastoma. Of 104 children with sporadic hereditary retinoblastoma born between 1945 and 1970, we studied the age of their parents at the birth and compared this age with the mean age of parents at the birth of their children during the same period in The Netherlands. The mean age of fathers at the birth of their children with sporadic hereditary retinoblastoma (33.7 years) was significantly higher than the mean age of fathers at the birth of their children in the general population (32.5 years) (P less than .05, one sided). Similarly, the mean age of mothers at the birth of their children with sporadic hereditary retinoblastoma (31.2 years) was significantly higher than the mean age of mothers at the birth of their children in the general population (29.5 years) (P less than .05, one sided). We further analyzed this parental age factor by measuring the relative risk of age groups and comparing the incidence of sporadic hereditary retinoblastoma in the various parental age groups with the incidence of sporadic hereditary retinoblastoma in the total population. Mothers 35 years of age or older had a relative risk of 1.7 to have a child with sporadic hereditary retinoblastoma compared with mothers in the population in general (P = .006, one sided). Similarly, fathers 50 years of age or older had a relative risk of 5.0 to have a child with sporadic hereditary retinoblastoma compared with fathers in the population in general (P = .04, one sided). No parental age effect was found in children with nonhereditary retinoblastoma. We conclude that a high paternal and a high maternal age are significant risk factors for sporadic hereditary retinoblastoma. 

Category: neoplasms
Genetics and biology of human ovarian teratomas. I. Cytogenetic analysis and mechanism of origin. One hundred and two benign, mature ovarian teratomas and two immature, malignant teratomas were karyotyped and scored for centromeric heteromorphisms as part of an ongoing project to determine the chromosomal karyotype and the genetic origin of ovarian teratomas and to assess their utility for gene-centromere mapping. Karyotypic analysis of the benign cases revealed 95 46,XX teratomas and 7 chromosomally abnormal teratomas (47,XXX, 47,XX,+8 [two cases], 47,XX,+15, 48,XX,+7,+12 91,XXXX,-13 [mosaic], 47,XX,-15,+21,+mar). Our study reports on the first cases of tetraploidy and structural rearrangement in benign ovarian teratomas. The two immature cases had modal chromosome numbers of 78 and 49. Centromeric heteromorphisms that were heterozygous in the host were homozygous in 65.2% (n = 58) of the benign teratomas and heterozygous in the remaining 34.8% (n = 31). Chromosome 13 heteromorphisms were the most informative, with 72.7% heterozygosity in hosts. The cytogenetic data indicate that 65% of teratomas are derived from a single germ cell after meiosis I and failure of meiosis II (type II) or endoreduplication of a mature ovum (type III); 35% arise by failure of meiosis I (type I) or mitotic division of premeiotic germ cells (type IV). 

Category: general pathological conditions
Auditory brain stem implant: effect of tumor size and preoperative hearing level on function. The auditory brain stem implant is an investigational device designed to provide hearing sensations to patients without functioning auditory nerves. We analyzed results from 17 implants in 15 patients to determine if tumor size or preoperative hearing level might be related to proper device function. We found no significant correlation between preoperative hearing level or tumor size and device function. We also found no significant correlation between preoperative hearing level and tumor size in these 15 patients. 

Category: nervous system diseases
Adenocarcinoma of the appendiceal stump. We have reported the case of a 58-year-old woman with nonspecific abdominal complaints in whom barium enema and subsequent colonoscopy showed a 3 cm lobulated adenocarcinoma within a villous adenoma arising from the appendiceal stump. Because such appendiceal malignancies have no specific clinical signs, symptoms, or radiologic features, preoperative diagnosis is extremely difficult, and colonoscopy may be required to clarify radiologically demonstrated irregularities. 

Category: general pathological conditions
Misplaced caval filter and subsequent pericardial tamponade. Use of the Greenfield filter for partial caval interruption is generally accepted as the most reliable mechanical method of pulmonary embolus prophylaxis. However, there have been reports of a variety of (usually nonfatal) complications. We report here the near-fatal complication of acute pericardial tamponade after misplacement of a Greenfield filter. Because of the filter's unusual location, retrieval required cardiopulmonary bypass, profound hyperthermia, and circulatory arrest. 

Category: cardiovascular diseases
Ataxia in myxoedema: a neurophysiological reassessment. In a long-standing case of myxoedema with ataxia and dysarthria, neurophysiological investigations were carried out to assess how much of the ataxic dysbasic syndrome depended on the slowness of mechanical contraction and how much resulted from primary cerebellar involvement. It was observed that the Achilles reflexogram showed a marked prolongation of contraction and relaxation time and that in both quadriceps and triceps surae mechanical percussion induced a marked myxoedema and prolonged relaxation time. The EMG of these muscles during voluntary contraction and stopping reaction detected an excessive recruitment of the antagonistic muscles, starting without any abnormal delay, a finding at variance with a typical cerebellar pattern. Post-urographic analysis gave a pattern of oscillations still within the normal range. These findings suggest that the gait alterations of our patient depended on the increase in muscle contraction time and the consequent excessive recruitment of the antagonists. 

Category: nervous system diseases
T-cell subsets with a naive phenotype are selectively decreased in the peripheral blood of patients with mycosis fungoides. Peripheral blood lymphocytes of 33 patients with histopathologic confirmation of mycosis fungoides and 27 healthy controls matched for age and sex were analyzed with a panel of monoclonal antibodies using both single and dual color immunofluorescence. Patients with mycosis fungoides had a significant reduction in the percentage of circulating T cells with a naive phenotype (i.e., CD4+2H4+ and CD4+Leu8+), as well as a significant reduction in the absolute numbers of circulating lymphocytes with the phenotype CD8+Leu8+ compared to the control cohort. The reduction in circulating naive T cells was found to occur irrespective of stage of disease, duration of disease, or mode of treatment. The depletion of circulating naive T cells may reflect increased conversion to memory T cells in the peripheral blood or skin. 

Category: neoplasms
Improved results with combined donor-specific transfusion (DST) and sequential therapy protocol. A combined DST-sequential CyA therapy protocol has been described that results in optimum graft survival for 1- and 2-haplotype mismatched living related donor-recipient combinations. In addition to the excellent graft survival obtained through 4 years, lower prednisone and CyA dosage levels are achieved with significantly decreased infection rates during the posttransplant period. 

Category: cardiovascular diseases
Fallopian tube cancer. The Roswell Park experience. Sixty-four patients with primary fallopian tube cancer treated at Roswell Park Memorial Institute from 1964 to 1987 underwent retrospective clinicopathologic review. In 40 patients fallopian tube cancer was the only primary, but in 24 patients primary fallopian tube cancer was part of a multifocal upper genital tract malignancy. Of the 40 patients with unifocal fallopian disease, the median survival was 28 months. Only 15% of patients were alive and disease free with follow-up ranging from 22 to 141 months (median, 90.5 months). Survival was not associated with stage of disease, tumor histology, grade, or depth of invasion in this series. Fourteen patients who received cisplatin-based chemotherapy were evaluable for response. Three patients (21%) responded; two complete and one partial. Twelve patients without clinical evidence of disease underwent second-look procedures, ten laparotomy and two laparoscopy. Four of ten second-look laparotomies were negative. Secondary debulking was done in three of four patients with gross disease, one of which had a negative third-look laparotomy. Negative laparotomy, second-look or third-look, was associated with improved survival (P = 0.016). One of the two laparoscopies was negative, but the patient recurred. In the remaining 24 patients cancer of the fallopian tube was part of a multifocal upper genital tract malignancy. In 12 patients tubal disease was invasive, and in 12, it was in situ. Separate primaries occurred in the ovaries (n = 20); uterus (n = 7); and cervix (n = 2). This represents 1.3% of ovarian malignancies treated at Roswell Park Memorial Institute during the study period. Fallopian tube cancer seems as virulent as ovarian cancer with few long-term survivors. It is frequently associated with other sites of upper genital tract malignancy. Second-look laparotomy is an important predictor of survival. Second-look laparoscopy may be useful if positive. 

Category: neoplasms
Initial therapy of patients with Wilson's disease with tetrathiomolybdate. Patients with Wilson's disease who present with acute neurological symptoms often become clinically worse when initially treated with penicillamine. Other available anticopper drug therapies do not appear to offer a solution to this treatment problem. We are developing and evaluating a new drug, ammonium tetrathiomolybdate for this purpose. Theoretically, tetrathiomolybdate has optimal properties, including an immediate blockade of copper absorption and the property of forming complexes with copper in the blood, rendering the copper nontoxic. In this article, we present results from six patients treated with tetrathiomolybdate for up to 8 weeks as initial therapy. None of the five patients who had presented with acute neurological symptoms worsened. Also presented are methods of assay, preliminary stability studies, and methods of evaluating therapeutic end points with respect to copper metabolism. 

Category: nervous system diseases
Interferon-alpha receptor expression and regulation in chronic hepatitis B virus infection. Interferon-alpha elicits antiviral and immunoregulatory activities by binding to specific receptors on the cell surface. In this study, binding characteristics of interferon-alpha to peripheral blood mononuclear cells in patients with chronic hepatitis B virus infection were studied using radioiodinated recombinant interferon-alpha 2b to determine interferon-alpha receptor numbers and dissociation constants. A single class of interferon-alpha receptor was demonstrated on peripheral blood mononuclear cells and mononuclear subsets. Peripheral blood mononuclear cells from patients with chronic hepatitis B virus infection (n = 20) and controls (n = 16) expressed a similar number of interferon-alpha receptors (484 +/- 175 vs. 511 +/- 168 sites/cell respectively, p = NS) with a similar dissociation constant (dissociation constant approximately 0.2 to 0.7 nmol/L). Expression of interferon-alpha receptors was similar in monocyte-enriched and lymphocyte-enriched fractions in both groups. Similar changes were observed in patients receiving alpha-interferon therapy. There was no correlation between interferon-alpha receptors expression and serum transaminase, serum HBsAg, serum HBV DNA, liver histological findings or the response to interferon-alpha therapy. After incubation of lymphocytes in vitro with interferon-alpha 2b (10 to 1,000 U/ml), interferon-alpha receptors number dropped by 42% to 80%, but this was associated with an increase in binding affinity (dissociation constant approximately 0.05 to 0.15 nmol/L) in both patients and controls. There was significant delay in the initial phase of receptor recovery in the patients with chronic hepatitis B virus infection compared with normal controls (days 1 and 2, p less than 0.05). 

Category: general pathological conditions
Cystic rheumatoid arthritis: description of a nonerosive form. In a study of patients with rheumatoid arthritis (RA), 9% (n = 70) were found to have a cystic form. At radiologic examination of these patients with cystic RA, the first abnormality seen consisted of periarticular intraosseous cysts without erosions. The cysts were distributed symmetrically, most often located at the proximal side of the joints and predominantly around the proximal interphalangeal, metacarpophalangeal, and wrist joints of the hands and the first interphalangeal and metatarsophalangeal joints of the feet. Computed tomographic scans showed the peripheral intraosseous location of the cysts. Magnetic resonance images showed that the cysts may contain fluid, inflamed synovia, or both. Cysts can be an important feature in the diagnosis of RA and a supplement to the criteria of the American Rheumatism Association. Osteoporosis, joint-space narrowing, and joint destruction occurred less frequently in patients with cystic RA than in patients with classic RA. Of the patients with cystic RA, 54% were male, and 50% were seronegative. This study is a supplement to and an enlargement on earlier descriptions of cyst predominance in RA. 

Category: neoplasms
Amputation or limb-lengthening for partial or total absence of the fibula. Thirty-two patients who had ablation of the foot by the Syme or Boyd technique for partial or total absence of the fibula, with subsequent fitting of a prosthesis, were compared with eleven patients who had lengthening of the lower limb by the Wagner method, to assess the long-term results of each procedure. The final results were evaluated on the basis of pain, limp, limb-length discrepancy, level of physical activity, and satisfaction of the patient. Of the thirty-two patients who had an amputation, twenty-eight (88 per cent) had a satisfactory result, compared with only six (55 per cent) of the eleven patients who had limb-lengthening. The amount of inequality between the lower limbs was classified as follows: Group I--the foot of the shorter extremity was at the distal third of the contralateral, normal limb, and the percentage of shortening was 15 per cent or less; Group II--the foot of the shorter extremity was at the level of the middle third of the contralateral, normal limb, and the percentage of shortening was between 16 and 25 per cent; and Group III--the foot of the shorter extremity was at the level of the proximal third of the contralateral, normal limb, and the percentage of shortening was greater than 26 per cent. Lengthening was best suited for patients in Group I who had stable hips, knees, and ankles and a plantigrade foot. Patients in Groups II and III were best served by ablation of the foot and fitting of a prosthesis. 

Category: general pathological conditions
Use of single internal mammary artery grafts in older patients. The indications for use of the internal mammary artery as a coronary bypass conduit in older patients are not clearly established. Between January 1985 and December 1988, 786 patients received single internal mammary artery grafts alone (717) or in conjunction with other procedures (69). Of these patients, 341 were 65 years of age or older (mean, 69.3 years). This group was compared with those patients less than 65 years of age (mean, 56.2 years). There were no significant differences between the two groups in the incidence of diabetes mellitus; the number of distal coronary anastomoses; left ventricular wall motion score; the frequency of isolated bypass procedures, reoperations, or emergent operations; or the durations of aortic clamping and cardiopulmonary bypass. The older group contained more women (34.9% versus 20.7%) and more patients with left main coronary artery disease (15.5% versus 9.4%) (p less than 0.01). No significant differences in the frequency of reoperation for hemorrhage, perioperative infarction, neurological deficits, requirements for intra-aortic balloon pumping, sternal wound infections, necessity for prolonged ventilatory support, or 30-day mortality (2.7% versus 3.2%) were observed. The length of postoperative hospitalization was greater in the older group (11.8 versus 10.2 days) (p = 0.02). Blinded histological examination of biopsies from 61 internal mammary artery grafts (34 patients less than 65 years, 27 patients greater than or equal to 65 years) showed no significant differences in luminal area or wall thickness. No significant degenerative changes were observed in either group. We conclude that the use of the internal mammary artery for bypass grafting in older patients is safe and may provide long-term benefit. 

Category: cardiovascular diseases
Differences in expression of cystic fibrosis in blacks and whites. The recent identification of the cystic fibrosis (CF) gene confirms that genetic heterogeneity occurs in CF. A three-base-pair deletion in exon 10 resulting in a loss of the phenylalanine residue at amino acid position 508 of the gene product, termed the CF conductance regulator protein, accounts for 70% of cases of CF in white subjects. However, this gene defect occurs in only 37% of affected blacks. Analysis of CF genes from American blacks has revealed a number of mutations, most of which are unique to that population. We therefore searched for potential differences in expression of CF between 24 black and 48 white patients with CF matched for birth date and gender. Black patients more frequently presented with only respiratory symptoms (38% vs 10%). Black patients had fewer hospitalizations for pulmonary exacerbations (2 vs 6.9), a better mean forced vital capacity (77% vs 62% of predicted), and higher chest roentgenogram scores (18.2 vs 14.4) than white patients. Complication rates were similar except for a higher incidence of hyponatremic dehydration (21% vs 2%) and peptic ulcer disease (13% vs 0%) in blacks. Survival time appeared to be longer in blacks, but the difference was not statistically significant. We conclude that phenotypic differences exist between black and white patients with CF, which may be due to the genetic heterogeneity between these two populations. 

Category: digestive system diseases
Binding to human jejunum of serum IgA antibody from children with coeliac disease. Jejunal histology and the presence of serum IgA antibodies (JAB) binding to human jejunum in vitro were studied in 139 children with severe malabsorptive symptoms. Among 33 children with confirmed coeliac disease (ESPGAN criteria), 13 (93%) of 14 sampled before starting on a gluten-free diet had JAB, none of 21 sampled had JAB while on a gluten-free diet of long duration, and 90% of 30 sampled during gluten challenge had JAB. 53 children had severe jejunal villous atrophy (probable coeliac disease): 71% of those younger than 2 years and 94% of those aged 2-18 years had JAB during gluten intake. JAB could not be detected in 53 disease control patients (normal jejunal histology) and in 3 coeliac disease patients with selective IgA deficiency. Simultaneous determination of antigliadin (AGA) and antiendomysium (EMA) levels, and gliadin and tissue absorption studies, showed that JAB and AGA are different, whereas JAB and EMA are probably identical. IgA JAB could be the target-organ-related autoantibodies in coeliac disease. 

Category: digestive system diseases
Fluosol: an oxygen-delivery fluid for use in percutaneous transluminal coronary angioplasty. Fluosol (20% intravascular perfluorochemical emulsion) is an oxygen-carrying emulsion used to deliver oxygen to ischemic myocardium during percutaneous transluminal coronary angioplasty (PTCA). Fluosol is composed of two perfluorochemicals, perfluorodecalin and perfluorotripropylamine. It has a high capacity for oxygen solubility, a low viscosity, and a small particle size. Following administration, the perfluorochemicals in fluosol are not metabolized. Rather, most are expired as gaseous particles through the lungs; the remainder are taken up by the organs of the reticuloendothelial system and later expired. When administered during balloon inflation in PTCA, fluosol preserves ventricular wall motion and global left ventricular ejection fraction. In addition, it minimizes ST segment changes and preserves cardiac output. Fluosol may be especially useful in patients who have poor contractile reserve, multivessel disease, or serious underlying illness. Other uses under investigation include limitation of myocardial infarct size and chemosensitization or radiosensitization of malignant tumors. Adverse effects secondary to the use of fluosol include ventricular arrhythmias, pruritus, bradycardia, chest pain, dyspnea, and increased respiratory rate. Fluosol must be thawed, admixed, warmed to body temperature, and oxygenated prior to intracoronary administration. The usual administration rate is 60 mL/min during each balloon inflation. 

Category: cardiovascular diseases
Kinetic abnormalities of the red blood cell sodium-proton exchange in hypertensive patients. The present study was designed to examine the kinetics of Na(+)-H+ exchange in red blood cells of normotensive and hypertensive subjects and its relation to the previously reported abnormalities in Na(+)-Li+ exchange. The Na(+)-H+ antiporter activation kinetics were studied by varying cell pH and measuring net Na+ influx (mmol/l cell x hr = units) driven by an outward H+ gradient. The Na(+)-Li+ exchange was determined at pH 7.4 as sodium-stimulated Li+ efflux. Untreated hypertensive patients (n = 30) had a higher maximal rate of Na(+)-Li+ exchange (0.43 +/- 0.05 versus 0.26 +/- 0.02 units, p less than 0.0003), a higher maximal rate of Na(+)-H+ exchange (62.3 +/- 6.2 versus 47 +/- 4 units; p less than 0.02), but a similar affinity for cell pH compared with normotensive subjects (n = 46). The cell pH activation of the Na(+)-H+ antiporter exhibited a lower Hill coefficient than that of normotensive subjects (1.61 +/- 0.12 versus 2.56 +/- 0.14; p less than 0.0001). This index of occupancy of internal H+ regulatory sites was found reduced in most of the hypertensive patients (73%) whether their hypertension was untreated or treated. Hypertensive patients with Na(+)-Li+ exchange above 0.35 units (0.68 +/- 0.057 units, n = 16) did not exhibit elevated maximal rates of Na(+)-H+ exchange (57.3 +/- 10 units, NS) in comparison with those with Na(+)-Li+ exchange below 0.35 units (66.4 +/- 7.6 units, n = 26), but both groups exhibited reduced Hill coefficients. Hypertensive patients with enhanced Na(+)-H+ exchange activity (more than 90 units) had normal maximal rates of Na(+)-Li+ exchange. 

Category: cardiovascular diseases