Melricflash/CW_MedAbstractsAlt huggingface dataset:
Frontal impairment and hypoperfusion in neuroacanthocytosis. Cerebral blood flow tomography, by xenon 133 inhalation or HMPAO (99mTc-d, l-hexamethyl-propylene amine oxime) technetium Tc 99m injection, revealed a severe hypoperfusion in both frontal lobes of a 40-year-old woman with confirmed neuroacanthocytosis. This finding occurred in conjunction with neuropsychological deficits consistent with selective frontal lobe dysfunction. This observation is the first documentation of this type of dementia in neuroacanthocytosis. 

Category: nervous system diseases
Bowel dysfunction in fibromyalgia syndrome. Fibromyalgia and irritable bowel syndrome frequently coexist. In this study, we utilized a previously validated self-administered questionnaire to assess the prevalence of symptoms of bowel dysfunction and irritable bowel syndrome in 123 patients with fibromyalgia as compared to 54 patients with degenerative joint disease (DJD) and 46 normal controls. Ninety (73%) of the fibromyalgia patients reported altered bowel function as compared to 20 (37%) DJD patients and none of the normal controls (P less than 0.001). Ninety-nine patients (81%) reported normal alternating with irregular bowel pattern, and 77 (63%) had alternating diarrhea and constipation. In contrast, only 24 (44%) of DJD patients and six (13%) of controls had regular alternating with irregular bowel pattern and only 12 (22%) of the DJD patients and none of the healthy controls had alternating constipation and diarrhea (P less than 0.01). Other bowel dysfunction complaints noted in the fibromyalgia group were abdominal gas (59%), nausea (21%), diarrhea (9%), and constipation (12%). Seventy-nine (64%) fibromyalgia patients reported frequent abdominal pain that was stress-related 47% of the time. Laxative use was frequent in the fibromyalgia group (19%) and absent in the other two groups. Fifty percent of fibromyalgia patients, compared to 28% of DJD patients, felt that their bowel complaints were worse during exacerbations of their joint disease (P less than 0.05). In conclusion, patients with fibromyalgia have a high prevalence of gastrointestinal complaints that should be carefully assessed. If the diagnosis of IBS is confirmed, appropriate treatment may improve patients' symptoms, although this approach requires further study. 

Category: general pathological conditions
Blood contacts during surgical procedures Operating room personnel are at risk for infection with blood-borne pathogens through blood contact. To describe the nature and frequency of blood contact and its risk factors, trained observers monitored a sample of operations performed by six surgical services at Grady Memorial Hospital, Atlanta, Ga, for 6 months. In 62 (30.1%) of 206 operations, at least one blood contact was observed. Of 1828 operating room person-procedures observed, 96 (5.3%) had 147 blood contacts (133 skin contacts [90%], 10 percutaneous injuries [7%], and four eye splashes [3%]). The mean number of blood contacts per 100 person-procedures was highest for surgeons (18.6). The frequency of percutaneous injury was similar among surgeons and scrub staff (mean, 1.2 per 100 worker-procedures for each group). Risk factors for surgeons' blood contacts were (1) performing a trauma, burn, or orthopedic emergency procedure (odds ratio [OR], 4.1; 95% confidence interval [CI], 2.0 to 8.7); (2) patient blood loss exceeding 250mL (OR, 2.1; 95% CI, 1.2 to 3.7); and (3) being in the operating room longer than 1 hour (OR, 3.3; 95% CI, 1.6 to 7.1). Of 110 blood contacts among surgeons, 81 (74%) were potentially preventable by additional barrier precautions, such as face shields and fluid-resistant gowns. Twenty-one (84%) of 25 blood contacts among surgeons in procedures in which all three risk factors were present were potentially preventable by additional barriers. Of 29 blood contacts among anesthesia and circulating personnel, 20 (69%) would have been prevented by glove use. For surgical procedures in which operating room personnel are at increased risk of blood contact, reevaluation of surgical technique, use of appropriate barrier precautions, and development of puncture-resistant glove materials are indicated. 

Category: digestive system diseases
Phase I clinical and pharmacologic study of intraperitoneal cisplatin and fluorouracil in patients with advanced intraabdominal cancer. Fluorouracil (5-FU) and cisplatin display marked therapeutic synergy in preclinical models and are effective in the treatment of a number of solid tumors when combined and administered intravenously (IV). Each drug has also been administered intraperitoneally (IP) and displays a favorable pharmacologic profile and acceptable clinical toxicity. We therefore undertook a phase I study to determine the feasibility and toxicity of combination IP chemotherapy with these agents. Thirty-one patients with histologically documented malignancy confined to the peritoneal space were treated with cisplatin 90 mg/m2 mixed with 5-FU in 2 L of lactated Ringer's solution and given IP for 4 hours every 28 days. Cohorts of at least three patients received starting 5-FU concentrations ranging from 5 mmol/L (1,300 mg in 2 L) to 20 mmol/L. The dose-limiting toxicity was neutropenia with a median granulocyte nadir of 156 cells per microliter occurring at a 5-FU dose of 20 mmol/L. Intrapatient escalation of the 5-FU dose was permitted and 15 cycles of chemotherapy were delivered at 5-FU concentrations greater than 20 mmol/L, the highest concentration being 30.7 mmol/L (8 g of 5-FU in 2L). Other toxicities included mild to moderate nausea during all cycles of therapy, vomiting in 54% of cycles, and diarrhea in 15% of cycles. Abdominal pain, renal dysfunction, peripheral neuropathy, and oral mucositis occurred infrequently and were not related to the 5-FU dose. Peritoneal fluid and plasma 5-FU concentrations were measured by high-performance liquid chromatography (HPLC) in selected patients. Mean peak plasma 5-FU concentrations ranged from 6.19 mumol/L to greater than 60 mumol/L, and peritoneal fluid to plasma 5-FU area under the curve (AUC) ratios ranged from 85 to 1,150. Nine of 15 patients with nonbulky disease had resolution of malignant ascites or at least a 50% reduction of peritoneal studding by tumor at repeat laparotomy. We conclude that combination IP chemotherapy with cisplatin and 5-FU is technically feasible and has acceptable clinical toxicity and a favorable pharmacologic profile. The recommended starting 5-FU dose for phase II trials is 3,900 mg mixed with 90 mg/m2 of cisplatin in 2 L of isotonic fluid. 

Category: digestive system diseases
Delayed-onset dystonia due to perinatal or early childhood asphyxia. We report 10 patients with delayed-onset dystonia associated with perinatal asphyxia and 2 associated with asphyxia in childhood. In the perinatal group, the mean age of onset was 12.9 years. Among these patients, dystonia continued to progress for a mean of 7 years, and as long as 28 years. These patients had moderate motor disability; none was wheelchair-bound, and thus their prognosis was better than that of the childhood-onset idiopathic torsion dystonias. The most frequently beneficial drugs were anticholinergics. Since some of these patients closely resembled cases of idiopathic torsion dystonia, the prior occurrence of asphyxia should be used as a criterion of exclusion for that diagnosis. 

Category: general pathological conditions
Myotonic heart disease: a clinical follow-up. We followed 37 patients with myotonic dystrophy for a mean of 6 years. Two developed atrial flutter or fibrillation, 6 developed a new bundle branch block, 1 developed complete heart block requiring a pacemaker, and another with progressive 1st-degree heart block and a widening QRS interval had a sudden death. Most patients had predictable, gradually progressive disease of their cardiac conduction system. We recommend that patients with progressive atrioventricular block or widening QRS interval due to myotonic heart disease have yearly ECGs and be questioned about syncope or presyncope to determine the need for a cardiac pacemaker. 

Category: general pathological conditions
Proliferation and DNA ploidy in malignant breast tumors in relation to early oral contraceptive use and early abortions. In 175 premenopausal breast cancer patients, a history of oral contraceptive (OC) use before 20 years of age was significantly associated with higher tumor cell proliferative activity, as indicated by a higher S-phase fraction (SPF), and a higher fraction of DNA aneuploid tumors, compared with later or never users (P = 0.05 and p = 0.01, respectively). The higher SPF among early OC users was apparent in patients with aneuploid tumors but not in patients with euploid tumors. Abortions (spontaneous or induced) before the first full-term pregnancy also were associated with a higher SPF compared with other young patients with breast cancer (P = 0.03). Adjusting for parity and abortions or OC use, respectively, an early OC use was associated with a 43% higher SPF and early abortions were associated with 49% higher SPF. Younger patients had a higher SPF and a higher frequency of aneuploid tumors, but this was found to be because the users of OC had a lower median age at diagnosis. Among never users, no significant age relationship was seen for SPF or the frequency of aneuploidy. For the DNA analyses there is a selection of patients with breast cancer with larger tumors, and therefore the conclusions drawn in this article may not be generalizable to patients with smaller primary tumors, e.g., cases diagnosed at breast cancer screening. The higher tumor proliferative activity and frequency of aneuploidy in early OC users are in line with previously reported findings of worse prognostic indicators and a worse survival in early users of OC compared with other young women with breast cancer. 

Category: neoplasms
Vascular abnormalities in epidermal nevus syndrome. We report a patient with epidermal nevus syndrome and right hemispheric infarct and review 3 others with neurologic manifestations best explained by ischemia or hemorrhage. Each had a significant vascular abnormality such as occlusion or blood vessel dysplasia. None had hemimegalencephaly. We hypothesize that underlying vascular dysplasia is the cause of the neurologic lesions in these patients. 

Category: cardiovascular diseases
Management of pseudarthrosis after arthrodesis of the spine for idiopathic scoliosis. Sixty-three first, second, or third-time repairs of one or more pseudarthroses were done in fifty-one patients who had had an arthrodesis for idiopathic scoliosis. Forty-five of the patients were female and six were male. The average age was 30.2 years. The indications for the sixty-three repairs were pain (twenty-five repairs), progression of the curve (sixteen), both pain and progression of the curve (twelve), and radiographic changes only (ten). Failure of the implant was identified before 27 per cent of the sixty-three procedures. The pseudarthroses were diagnosed an average of 2.8 years after the initial arthrodeses. Sixty-eight per cent of the defects were visible on plain radiographs preoperatively and 32 per cent were identified at operation. During the time between the original arthrodeses and the repairs of the pseudarthroses, the scolioses increased by a mean of 7 degrees and the kyphoses, by a mean of 10 degrees. Harrington distraction was the most commonly used instrumentation (twenty-six [41 per cent] of the sixty-three procedures), and autogenous iliac bone was the most commonly used material for the graft (thirty-three [52 per cent] of the procedures). 

Category: nervous system diseases
Transforming function of proto-ras genes depends on heterologous promoters and is enhanced by specific point mutations [published erratum appears in Proc Natl Acad Sci U S A 1991 May 1;88(9):4059] Based on transfection into cells in culture or natural transduction into retroviruses, proto-ras genes seem to derive transforming function either from heterologous promoters or from point mutations. Here we ask how such different events could achieve the same results. To identify homologous regulatory elements, about 3 kilobases of rat DNA upstream of the first untranslated proto-Ha-ras exon was sequenced. Surprisingly, the sequence shares at -1858 a homology of 148 nucleotides with Harvey (Ha) sarcoma virus, 5' of viral ras, signaling possibly a second untranslated proto-Ha-ras exon. In addition the sequence contains a perfect repeat of 25 CA dinucleotides at -2655. A retroviral promoter, even from upstream of the poly(CA), conferred transforming function on proto-Ha-ras and increased transcription greater than 100-fold compared with that of unrearranged proto-ras. Point mutations were not necessary for transforming function of rat and human proto-Ha-ras genes with retroviral promoters but did enhance it greater than 10-fold. A unifying hypothesis proposes that proto-ras genes depend on high expression from heterologous promoters or enhancers for transforming function, which is modulated by ras point mutations. The hypothesis makes two testable predictions. (i) Unrearranged proto-ras genes with point mutations, which occur in some cancers, have no transforming function. Indeed, tumors with mutated proto-ras genes, even those that also lack hypothetical tumor-suppressor genes, are indistinguishable from counterparts with normal proto-ras genes. (ii) Proto-ras genes in transfected cells derive transforming function from heterologous promoters or enhancers acquired via illegitimate recombination from vector DNAs and particularly from viral helper genes that must be cotransfected for transformation of primary cells. Indeed, expression of exogenous proto-ras genes in cells transformed by transfection is as high as for viral ras genes and is much higher than in the cells of origin. 

Category: neoplasms
A longitudinal study of respiratory symptoms in a community population sample. Correlations with smoking, allergen skin-test reactivity, and serum IgE Chronic cough and/or phlegm, wheeze in the absence of colds, and rhinitis attributed to allergies are three of the most common respiratory symptoms encountered in community populations. In this study, we have determined the prevalence of these complaints in a random population sample (n = 1,109) using standardized questionnaires at two points in time, eight years apart. Cross-sectional prevalence and changes in symptom occurrence have been correlated with smoking status, allergen skin test reactivity, and total serum IgE levels. Our objective was to determine the individual and combined influence of these three variables on symptom prevalence. Initially, 19.2 percent of the population admitted to wheeze, 17.9 percent to cough, and 44.1 percent to allergic rhinitis. Cough and wheeze prevalence changed little over the eight-year period, while rhinitis increased 11 percent by the second survey. The occurrence of chronic cough was strongly correlated with smoking, and was not further influenced by either allergen skin reactivity or IgE level. Conversely, rhinitis prevalence was related to skin test reactivity with no additional association with smoking or IgE level. The occurrence of wheeze in the absence of colds was associated with both smoking and allergen skin reactivity. Among smokers, the prevalence was over 30 percent and was similar in both skin test positive (STP) and skin test negative (STN) individuals. However, on both surveys, STP ex-smokers and nonsmokers had significantly more wheeze than those who were STN. While the prevalence of wheeze in STN nonsmokers was low (6.8 percent), an IgE-wheeze relationship was also suggested on the second survey. In addition to these cross-sectional symptom relationships, changes in either smoking status or allergen skin reactivity during the study period were associated with changes in the prevalence of each symptom. 

Category: general pathological conditions
Renovascular hypertension. Difficulties in diagnosis and treatment. Renovascular hypertension is not easily identified clinically, and most tests are not totally reliable in detecting its presence. The condition may be due to fibromuscular dysplasia or congenital anomalies in patients younger than age 30 or to atherosclerosis in patients older than 50. The goal of treatment is to lower blood pressure and improve or maintain renal function. Cure or improvement of hypertension is more likely in patients with fibromuscular dysplasia than in those with atherosclerosis. Interventional procedures include percutaneous transluminal angioplasty, surgical revascularization, and nephrectomy. 

Category: cardiovascular diseases
Unusual cerebral manifestations in hereditary fructose intolerance. Five children with hereditary fructose intolerance developed symptoms of neurological impairment. In three of them, neurological involvement was related to the acute hepatic toxicity of fructose (hypoglycemia, abnormal coagulation, cardiovascular collapse); in the other two, such a relationship could not be demonstrated. Neurological impairment is not classic in hereditary fructose intolerance, but its occurrence in the acute phase of the disease is possible and does not constitute an argument against the diagnosis. 

Category: nervous system diseases
Diffuse hemangiomatosis of the spleen: splenic hemangiomatosis presenting with giant splenomegaly, anemia, and thrombocytopenia. In an elderly patient with oligosymptomatic giant splenomegaly, clinical and laboratory data were nondiagnostic, while nonhomogeneous splenic enlargement was the only finding detected by imaging procedures. Splenectomy was performed and diffuse hemangiomatosis of predominantly capillary-type found. The failure of imaging techniques to even hint at the nature of the underlying disorder is comprehensible in view of the organ being essentially replaced in toto by the abnormal vascular channels. Diffuse splenic hemangiomatosis, a rare condition, may cause hypersplenism, and its diagnosis may be elusive because of misleading patterns on imaging. 

Category: neoplasms
Characterization of liver-associated natural killer cells in patients with liver tumors. The existence of a marginal lymphocyte population in rat liver sinusoids has already been demonstrated using the sinusoidal lavage method. We used the same technique to study the lymphocyte population in human liver obtained ex vivo after partial hepatectomy for benign or malignant tumors and compared it with peripheral and portal blood lymphocyte populations. Percentages of lymphocyte surface phenotypes were evaluated by flow cytometry. The lymphocyte population obtained from human liver is mainly made up of CD56+ (35%) cells. This percentage is three times greater than that found in peripheral and portal blood. Two-color flow cytometry analysis showed that within the CD56+ liver cell population, at least three distinct subsets could be found: (a) CD3+/CD56+/CD16-; (b) CD3-/CD56+/CD16-; and (c) CD3-/CD56+/CD16+. Although these subsets were also present in peripheral and portal blood, the percentage distribution was completely different because most CD56+ cells in peripheral and portal blood belonged to the CD3-/CD56+/CD16+ subset. These results show the existence of a heterogeneous natural killer cell population in human livers with tumors. The functional significance of this heterogeneity still needs to be explained. 

Category: neoplasms
Axial loading injuries to the middle cervical spine segment. An analysis and classification of twenty-five cases. Injuries to the cervical spine at the C3-C4 level involving the bony elements, intervertebral disks, and ligamentous structures are rare. We present 25 cases of traumatic C3-C4 injuries sustained by young athletes and documented by the National Football Head and Neck Injury Registry. Review of the cases reveals that the response of energy inputs at the C3-C4 level differ from those involving the upper (C1-C2) and lower (C4-C5-C6-C7) cervical segments. Specifically, the C3-C4 lesions appear to be unique with regard to the infrequency of bony fracture, difficulty in effecting and maintaining reduction, and a more favorable recovery following early, aggressive treatment. In the majority of instances, injury at this level results from axial loading of the cervical spine. Lesions were distributed into specific categories: 1) acute intervertebral disc herniation (N = 4), 2) anterior subluxation of C3 on C4 (N = 4), 3) unilateral facet dislocation (N = 6), 4) bilateral facet dislocation (N = 7), and 5) fracture of vertebral body C4 (N = 4). Analysis of these 25 cases suggests that traumatic lesions of the cervical spine in general can be classified as involving the upper (C1-C2), middle (C3-C4), or lower (C4-C7) segments. This is based on our observations from this series that C3-C4 lesions 1) generally do not involve fracture of the bony elements; 2) acute intervertebral disc herniations are frequently associated with transient quadriplegia; 3) reduction of anterior subluxation of C3 on C4 is difficult to maintain; 4) reduction of unilateral facet dislocation is difficult to obtain by skeletal traction and is best managed by closed manipulation and reduction under general anesthesia; and 5) reduction of bilateral facet dislocation is difficult to obtain by skeletal traction and is best managed by open methods. The more favorable results observed in this series of immediate reduction of both unilateral and bilateral facet dislocations deserves emphasis. In two cases of unilateral facet dislocation reduced within 3 hours of injury and subsequently fused anteriorly, significant neurologic recovery occurred. The other four patients, two who underwent an open reduction and laminectomy and two treated closed with skeletal traction, remained quadriplegic. In the four instances of bilateral facet dislocation where reduction was achieved by either closed or open methods, although there was no neurologic recovery, all four patients survived their injuries. However, the three patients who were not successfully reduced died. 

Category: nervous system diseases
Leukemia initiated by hemopoietic stem cells expressing the v-abl oncogene. We report a mouse model with which to study leukemogenesis initiated by a specific genetic change introduced into a primary lymphoid-myeloid pluripotent stem cell. Fetal liver hemopoietic cells were infected with a high titer of helper-free Abelson murine leukemia virus (A-MuLV) and were used to reconstitute lethally irradiated mice. Two weeks later, progenies of a single primitive hemopoietic stem cell carrying a specifically integrated A-MuLV proviral DNA could be detected in both colony-forming units in spleen and myeloid colony-forming cells in the bone marrow. Beginning at 3 weeks after transplantation, the recipients developed elevated leukocyte counts, splenomegaly, and increase of blast cells in the peripheral blood. Multiple clones of A-MuLV-infected cells were infused into each recipient. However, in the same animal, DNA extracted from various affected organs and from factor-independent lymphoid and myeloid immortalized cells all contained an identical, specifically integrated proviral genome. The A-MuLV-infected stem cells differentiated into various lineages of hemopoietic cells. Our data show that the expression of the v-abl oncogene in a primary lymphoid-myeloid hemopoietic stem cell directly initiates leukemogenesis by stimulating factor-independent growth. The monoclonal-type disease development seen in these animals may require the occurrence of an additional genetic event. 

Category: general pathological conditions
Acute hypercalcemic crisis after an open heart operation. Acute hyperparathyroidism developed in a previously normocalcemic 64-year-old woman during the first week after a coronary operation. Prolonged QT interval in the electrocardiogram and hypercalcemia were documented on the fourth postoperative day. Neck exploration on the fifth postoperative day revealed a lower right parathyroid adenoma. Parathyroidectomy resulted in rapid and dramatic improvement of the clinical picture and normalization of laboratory values. 

Category: general pathological conditions