shukoor65/lab-rag-model huggingface dataset:
Short-bowel syndrome in children. Quality of life in an era of improved survival. A number of disorders in childhood can result in short-bowel syndrome (small bowel length, less than 100 cm). Improved care has increased survival in patients with short-bowel syndrome, but the quality-of-life factors associated with such improved survival have not been examined, to our knowledge. Sixteen consecutive pediatric patients with short-bowel syndrome (bowel length range, 22 to 98 cm) were followed up for 2 to 10 years. The original diagnoses were as follows: necrotizing enterocolitis (n = 6), multiple intestinal atresias (n = 4), extensive aganglionosis (n = 2), meconium peritonitis (n = 2), and midgut volvulus (n = 2). The range of initial hospitalization was from 62 to 395 days, and 13 of 16 patients have required readmission (two to 14 times). All patients required multiple operations (range, two to 14 operations), including combinations of venous access, adhesiolysis, tapering enteroplasty, reversed intestinal segments, and pull-through procedure. Nine of 16 patients received home total parenteral nutrition, and 12 of 16 patients required home elemental diets, usually via pump feedings. Fifteen patients (94%) survived. Two survivors are deaf, and one of these has mild developmental delay. Seven survivors (age range, 6 to 10 years) attend a regular school, four while receiving total parenteral nutrition or an elemental diet. Ten of 15 survivors are off all nutritional support (including the child with a 22-cm small bowel), with four others weaning. The presence or absence of an ileocecal valve did not affect outcome. Modern nutritional support methods provide excellent survival and quality of life for children with short-bowel syndrome.
Diagnostic accuracy of exercise thallium-201 single-photon emission computed tomography in patients with left bundle branch block. Recent reports have proposed that abnormal apical or anterior wall perfusion with exercise thallium-201 imaging may increase diagnostic accuracy for disease of the left anterior descending artery in patients with left bundle branch block (LBBB). To evaluate these suggestions, 83 patients with LBBB who underwent thallium-201 single-photon emission computed tomography and coronary angiography within an interval of 3 months were retrospectively reviewed. There were 59 men and 24 women aged 33 to 84 years (mean 65). Myocardial perfusion to the apex, anterior wall and anterior septum were scored qualitatively by consensus of 2 experienced observers and by quantitative analysis in comparison with a normal data base. The sensitivity, specificity and accuracy of perfusion defects in these segments were then expressed according to angiographic findings. Significant stenosis of vessels within the left anterior descending artery territory was present in 38 patients. By receiver-operator characteristic analysis, a fixed or reversible defect within the apex by the qualitative method was the best criterion for coronary artery disease. However, although highly sensitive (79 and 85% by the qualitative and quantitative methods, respectively), an apical defect was neither specific (38 and 16%, respectively), nor accurate (57 and 46%, respectively). Perfusion abnormalities in the anterior wall and septum were also of limited diagnostic accuracy. Thus, modified interpretative criteria in patients with LBBB are not clinically useful in the assessment of left anterior descending artery disease.
NIT-1, a pancreatic beta-cell line established from a transgenic NOD/Lt mouse. NOD/Lt mice harboring a hybrid rat insulin-promoter/SV40 large T-antigen gene spontaneously develop beta-cell adenomas. NIT-1 is a pancreatic beta-cell line established from one of these transgenic mice. Immunocytochemical staining of passage 18 cells showed most contained insulin, with less than 5% containing glucagon, and none containing pancreatic polypeptide or somatostatin. Glucagon content radioimmunoassayed in cell extracts was only 0.27% of the insulin content. Two-hour insulin secretion at 16.5 mM glucose was 638 ng/10(6) cells (41% of intracellular content) compared to only 1.3 ng glucagon (32% of intracellular content). Stimulated insulin secretion was consistently observed in response to 11 and 16.5 mM glucose between passages 11 and 19. At passage 19, both theophylline and tolbutamide stimulated insulin secretion at 5.5 mM glucose. Northern-blot analysis confirmed high levels of insulin mRNA but only trace glucagon mRNA and undetectable somatostatin mRNA. Interferon-gamma (IFN-gamma)-induced MHC class I RNA expression was correlated with markedly increased antigen expression at the cell surface. Similarly, a MHC-linked "occult" class I-like antigen detected by Cr release assay only after exposure of standard NOD/Lt islet cells to IFN-gamma was strongly induced by IFN-gamma in NIT-1 cells. Cell surface MHC class II antigen was not constitutively expressed on NIT-1 cells and could not be detected after IFN-gamma incubation, despite demonstration of IFN-gamma-induced Aa, Ab, and Li invariant-chain RNA transcripts. Similarly IFN-gamma induction of intercellular adhesion molecule 1 (Icam-1) transcripts was not accompanied by demonstrable cell surface expression of ICAM-1 antigen.
Niemann-Pick type B disease. Identification of a single codon deletion in the acid sphingomyelinase gene and genotype/phenotype correlations in type A and B patients. Types A and B Niemann-Pick disease both result from the deficient activity of the lysosomal hydrolase, acid sphingomyelinase (E.C. 3.1.4.12). Type A Niemann-Pick disease is a severe neurodegenerative disorder of infancy which leads to death by three years of age, whereas Type B disease has a later age at onset, little or no neurologic involvement, and most patients survive into adulthood. To investigate the molecular basis for the remarkable phenotypic heterogeneity, the nature of the mutations causing Type B Niemann-Pick disease in Ashkenazi Jewish patients was determined. The entire acid sphingomyelinase coding region from an Ashkenazi Jewish Type B patient was polymerase chain reaction-amplified, subcloned, and completely sequenced. A three-base deletion was identified of nucleotides 1821-1823 in the cDNA which predicted the removal of an arginine residue from position 608 of the acid sphingomyelinase polypeptide (delta R608). The other cDNA clones from this patient had the R496L mutation previously identified in Type A Niemann-Pick disease patients. Both Ashkenazi Jewish Type B patients were heteroallelic for the delta R608 mutation, whereas this allele was not present in 15 unrelated non-Jewish Type B patients, with the notable exception of one mildly affected patient of Arabic descent who was homoallelic for the delta R608 mutation. These results indicate that the delta R608 mutation predicts the Type B Niemann-Pick disease phenotype, even in the presence of the R496L Type A allele, thereby providing the first genotype/phenotype correlation for this lysosomal storage disease. Although only two patients have been studied, it appears that the delta R608 mutation occurs frequently in Type B Niemann-Pick disease patients of Ashkenazi Jewish descent.
Development of a highly specific and sensitive molecular probe for detection of cutaneous lymphoma. Using DNA from formalin-fixed paraffin-embedded tissue of a patient with mycosis fungoides, a highly specific and sensitive molecular probe for the malignant lymphoid cells of this patient was developed. Polymerase chain reaction (PCR) was used to selectively amplify the region of the rearrangement of TCR-gamma genes. Randomly inserted nucleotides between rearranged segments (N-region) were used as a specific marker for the malignant lymphoid clone of this patient. Clonal cells in paraffin-embedded tissue of previous and later biopsies were detected. This technique provides a new and unique tool for retrospective detection of early evolving cutaneous lymphoma, as well as for detection of minimal residual and systemic disease.
Intraoperative transesophageal Doppler color flow imaging used to guide patient selection and operative treatment of ischemic mitral regurgitation. BACKGROUND. Intraoperative transesophageal Doppler color flow imaging (TDCF) affords the opportunity to assess mitral valve competency immediately before and after cardiopulmonary bypass (CPB). The purpose of this study was to assess the utility of TDCF to assist in the selection and operative treatment of ischemic mitral regurgitation (MR). METHODS AND RESULTS. Two hundred forty-six patients undergoing surgery for ischemic heart disease were prospectively studied. All had preoperative cardiac catheterization. Catheterization and pre-CPB TDCF were discordant in their estimation of MR in 112 patients (46%). Compared with patients in whom both techniques agreed in estimation of MR, patients with discordance in MR were more likely to have had unstable clinical syndromes at the time of catheterization (79% versus 40%, p less than 0.05) or to have received thrombolytics (16% versus 8%, p less than 0.05). Pre-CPB TDCF resulted in a change in the operative plan with respect to the mitral valve in 27 patients (11%). Because less MR was found by TDCF than catheterization, 22 patients had only coronary bypass grafting when combined coronary bypass and mitral valve surgery had been planned. Because more MR was found by TDCF than catheterization, five patients had combined coronary bypass and mitral valve surgery when coronary bypass alone had been planned. Unsatisfactory results noted by TDCF following mitral valve surgery in five patients resulted in immediate corrective surgery. Cox regression analysis identified residual MR at the completion of surgery to be an important predictor of survival (chi 2 = 21.4) after surgery--more important than patient age (chi 2 = 8.3) or left ventricular ejection fraction (chi 2 = 5.3). CONCLUSIONS. These results indicate that TDCF is useful in guiding patient selection and operative treatment of ischemic MR and that in such patients, intraoperative TDCF should be performed routinely.
Special considerations in the physical exam of older patients. For diagnostic utility, aspects of the standard physical examination are modified for elderly patients. Special attention is paid to problems that interfere with function, such as gait disorders, incontinence, and hearing and visual loss. Certain findings in cardiac and pulmonary examinations of the elderly require different interpretations. The examination should include the head and neck, chest, back, abdomen, breasts and pelvic area in women, prostate in men, extremities, and skin, as well as mental status. A number of diagnostic maneuvers are used to distinguish changes of normal aging from those associated with disease.
Thumbtack use for control of presacral bleeding, with description of an instrument for thumbtack application. There is well-known difficulty in controlling hemorrhage from presacral vessels. There are reports on the use of thumbtacks to secure hemostasis in the face of presacral hemorrhage. This communication reports the successful use of thumbtacks to establish presacral hemostasis and describes a simple instrument designed to afford easier and more precise anterior sacral application of thumbtacks. The instrument is easy to make. It was devised because of difficulty encountered with manual placement of hemostatic thumbtacks and the absence of totally satisfactory delivery by any of the standard surgical instruments available.
Suburethral paraganglioma. Masses presenting beneath the urethra are not uncommon. Besides ureteral diverticula, which account for the vast majority of these masses, a multiplicity of heterogeneous benign and malignant tumors have been encountered in this region. To the best of our knowledge, we report the first case of a neuroendocrine tumor, a paraganglioma, discovered in the anatomical compartment between the urethra and vagina. When evaluating a patient with a suburethral mass, it is important to consider paraganglioma as part of the differential diagnosis because unwitting surgical removal of a functional catecholamine-secreting tumor may precipitate a severe hypotensive episode and/or death.
The use of anterior Caspar plate fixation in acute cervical spine injury. Optimal management of cervical cord injury in the presence of documented instability and/or compression of neural elements remains a controversial topic. Surgery and internal stabilization of cervical spine fracture/dislocations are effective and well accepted, but controversy exists on the relative merits of the anterior versus the posterior approach as well as the optimal timing of surgical intervention. We report our experience with the Caspar technique and instrumentation for anterior stabilization in 54 patients for acute cervical spine injury. Our series consists of 38 male and 16 female patients whose ages ranged from 16 to 68 years, with a mean age of 29.2 years. Thirty-two of these patients had complete neurological sensory/motor deficits at the time of presentation, eight were neurologically intact, and 14 had preservation of some motor and sensory function. All 54 patients had radiographic evidence of posterior instability as well as anterior disruption of either a vertebral body or intervertebral disk. We found that "early" intervention (less than 24 hours after injury) was performed frequently in the neurologically compromised patients. Twelve of the 22 patients undergoing surgery less than 24 hours after admission regained significant neurological function, with 13 of 22 developing postoperative complications. In the "delayed" group (surgery more than 24 hours after injury, mean 14.3 days), 14 patients experienced postoperative complications, with 15 of 24 demonstrating neurological improvement. The eight patients who were intact did uniformly well. There was no mortality during the follow-up. All 54 patients showed a solid fusion (clinically and radiologically) within 6 months of surgery. In two cases the plates had to be removed, without risking the fusion. Our experience suggests that although anterior cervical fusion and Caspar plating remain appropriate for patients with documented anterior compromise of the canal, it should not substitute for more traditional posterior stabilization procedures. Because this route has the potential for more serious complications, it should be reserved for the cases in which anterior decompression is deemed necessary or posterior fusion was unsuccessful. With appropriate selection of patients, no adverse effect of early surgery was demonstrated. In fact, neurologically compromised patients had the benefits of increased ease of patient care and early transfer to rehabilitation.
Spatial contrast sensitivity is reduced in bilateral Parkinson's disease. We studied the contrast sensitivity functions of 41 patients with idiopathic Parkinson's disease (PD) with a wide range of parkinsonian symptomatology (Hoehn and Yahr stages 1 to 4) and 22 age-matched control subjects in a parametric design. Results demonstrated reduced contrast sensitivity in PD patients but only in those patients who had progressed beyond Hoehn and Yahr stage 1. Furthermore, there were deficits in contrast sensitivity related to the severity of PD.
Proliferation of normal breast epithelial cells as shown by in vivo labeling with bromodeoxyuridine. The proliferative activity of normal acinar and ductal breast epithelial cells was studied by in vivo labeling with 5-bromodeoxyuridine (BrdUrd) in 26 cases with concurrent breast carcinoma. The BrdUrd-labeled cells were recognized in histologic sections of paraffin-embedded tissue, using an anti-BrdUrd antibody and an immunoperoxidase reaction. The percentage of BrdUrd-labeled cells showed great variability for both acinar (0% to 2.66%; mean, 0.70%; standard deviation [SD], 0.80%) and ductal cells (0% to 1.99%; mean, 0.51%; SD, 0.57%). The fraction of proliferating epithelial cells declined with the age of the patients and was significantly higher in premenopausal women (1.16% +/- 0.85% for acinar and 0.94% +/- 0.60% for ductal cells) as compared with the postmenopausal women (0.27% +/- 0.46% for acinar and 0.17% +/- 0.22% for ductal cells), P less than 0.01 for acinar and P less than 0.001 for ductal cells, respectively. In some patients, great variability in distribution of proliferating acinar and ductal cells among different lobules and ducts was observed. No difference was found in the number of proliferating acinar and ductal cells situated near or far from their corresponding tumors. No correlation was seen between cell proliferation of normal acinar or ductal cells and cell proliferation of the respective tumors.
The safety of intraaortic balloon pump catheter insertion through suprainguinal prosthetic vascular bypass grafts. To determine the safety of intraaortic balloon pump catheter insertion for critical coronary ischemia through suprainguinal prosthetic bypass grafts, we examined our experience of 19 intraaortic balloon pumps placed through grafts in 17 patients by means of surgical exposure (8) or the percutaneous (11) approach. Fourteen intraaortic balloon pumps were placed through matured grafts at a time remote (2 to 13 years; mean, 7 years) from their vascular bypass surgery. Five were inserted through nonmatured grafts during the same hospitalization as their vascular reconstructive surgery (1 to 12 days; mean, 4 days). One patient, a day after an aortoiliac bypass, died during an urgent, surgical intraaortic balloon pump insertion for cardiogenic shock. All other patients had prompt reversal of their cardiac ischemia. Decreased limb perfusion developed during use of the intraaortic balloon pump in three patients, all of whom had their intraaortic balloon pump placed percutaneously through mature grafts. Two of these required surgical thrombectomy. Ten intraaortic balloon pump insertions in eight patients survived the hospitalization and were followed for a mean of 24 months (range, 2 weeks to 64 months). No localized groin or graft infections were identified. No bleeding complications or pseudoaneurysms occurred. Thus in patients with unstable, severe, cardiac disease, intraaortic balloon pumps can be safely placed through indwelling suprainguinal bypass grafts.
Vasculitis. A practical approach to management. Vasculitis encompasses a wide variety of diseases. Because diagnosis may be difficult, a careful evaluation is essential, including a detailed patient history, thorough physical examination, and appropriate laboratory studies. Diagnosis is based on clinicopathologic features that permit identification of the condition. Biopsies are often necessary to confirm a diagnosis. It is important to accurately categorize the vasculitic disorders, since prompt, aggressive therapy with potentially toxic drugs is necessary to avoid irreversible organ system dysfunction.
Family and peer issues among adolescents with spina bifida and cerebral palsy. Increasingly, children with disabling conditions are surviving through childhood into adolescence and beyond. This places increased importance on understanding adolescent, family, and peer relationships, which are critical to healthy development among adolescents in general and to transitions to successful adulthood among youths with physical disabilities in particular. The purpose of this study was to identify the patterns of family and peer interactions. One hundred two youths with spina bifida and 60 adolescents with cerebral palsy between the ages of 12 and 22 completed written psychological measures as well as an in-depth structured interview. Approximately 90% of youths with spina bifida and cerebral palsy resided at home, of whom 80.4% and 72% lived with both parents. Almost without exception, parental relationships were defined as positive, with no difference of attitude noted toward the mother and father. Relationships with parents for teenagers with spina bifida were marked by high levels of dependence on parents for personal care such as bowel programs and, for both groups, low levels of responsibility at home and lack of discussion with parents about sexuality or menstruation. Although friends were viewed as very important by most respondents (approximately 83%), relationships were characterized by extremely limited out-of-school contacts, negligible participation with organized social activities, and a primary orientation toward sedentary activities. Finally, regarding heterosexual relationships, whereas 44.1% of respondents with spina bifida and 54.2% of respondents with cerebral palsy said their friends dated, only 14.7% and 28.3% of study participants indicated that they had ever been on a date and, even for those teenagers, heterosexual social contacts were infrequent.
Barrett's esophagus and adenocarcinoma of the esophagus and gastroesophageal junction. Since 1985, 57 patients with adenocarcinoma of the esophagus and gastroesophageal (GE) junction have undergone surgical resection. In this group, 16 of the tumors arose in a Barrett's esophagus. There was a significant predilection toward white men above the age of 55 (15/16; 94%) in this subgroup. The mean proximal extent of abnormal columnar involvement was 5.4 cm above the gastroesophageal junction (range 2.5 to 11 cm). The mean location of the neoplasm centered in the distal esophagus 1.8 +/- 0.5 cm above the gastroesophageal junction. During the same time period, 30 patients with Barrett's esophagus were seen without associated adenocarcinoma. There were no statistical differences in the proximal extent of columnar involvement or the presence of reflux symptoms between the two groups. There were no significant differences in age, smoking history, and alcohol consumption between patients with benign or malignant Barrett's esophagus as compared to those with adenocarcinoma of the gastroesophageal junction not associated with Barrett's mucosa. The marked male predominance seen in the group with malignant Barrett's esophagus was in contrast to the benign cases (16/30; 53%) but was similar to the adenocarcinoma group, without recognized Barrett's esophagus (38/41; 93%). The mean location of the tumor in the latter was 0.9 +/- 1.2 cm above the gastroesophageal junction and was comparable to the location in the group with Barrett's adenocarcinoma. The 4-year survival rate of patients in the non-Barrett's adenocarcinoma group is approximately 30%. Of those with Barrett's adenocarcinoma, the present 4-year survival rate is 60%. The demographic and morphometric similarities between the Barrett's and non-Barrett's adenocarcinoma groups may be of primary importance in determining the true clinical prevalence of Barrett's adenocarcinoma. Our findings suggest that the sensitivity of endoscopic surveillance may be improved if biopsy specimens are concentrated within the distal 3 cm of the esophagus and the esophagogastric junction. Finally, the reason for the current difference in survival between the Barrett's and non-Barrett's adenocarcinoma groups is uncertain but may be related to endoscopic surveillance permitting earlier diagnosis and treatment.
Selective peripheral denervation for torticollis: preliminary results Herein we report the preliminary results in nine patients who have undergone selective peripheral denervation for spasmodic torticollis and have been followed up for at least 13 months. All patients had improvement immediately after surgical intervention, and the results have been maintained in five patients. In one patient who had recurrent torticollis, a second procedure in conjunction with injection of botulinum toxin has produced substantial improvement; however, follow-up was brief (6 months). No surgical complications occurred. We believe that selective peripheral denervation is safe and that it can benefit patients with torticollis who have not responded to other types of therapy. These favorable results confirm other published reports on the efficacy of selective peripheral denervation. Long-term follow-up, however, is necessary for determining the role of this procedure in the management of torticollis.