[A surprising calvarial sample].
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Biomedical subjects
Publications and source records attributed to D Orenstein.
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Pituitary stimulation tests are widely used to explore hypophyseal adenomas. There are few disadvantages, although a few cases of pituitary necrosis have been published. We report a new case with a dramatic outcome. A 30-year-old man with clinical signs of acromegalia and major visual disorders was found to have a voluminous macro-adenoma of the pituitary gland. Thirty minutes after beginning the stimulation test, the patient complained of major headache and experienced persistant vomiting for several hours. Brain magnetic resonance imaging the next day did not reveal evidence of pituitary necrosis. Sudden onset coma occurred one hour later. The CT scan demonstrated hemorrhagic necrosis of the adenoma. The patient died despite emergency surgery. Due to the risk of hemorrhagic necrosis of a pituitary adenoma, baseline assays may be sufficient for diagnosis in patients with clinical signs highly suggestive of pituitary oversecretion, especially when a voluminous tumor is involved. Rigorous clinical surveillance is required after stimulation tests.
Published articles on the radiologic aspects of Proteus syndrome are sparse. This report highlights the features of this disease with specific attention to the serious pulmonary manifestations that may occur at an early age. Two cases of Proteus syndrome and severe lung disease are presented, with complete autopsy in one case and correlative surgical pathologic data in the other. Multiple superficial and visceral vascular abnormalities were present in both cases. Both patients developed rapidly progressive diffuse cystic emphysematous pulmonary disease leading to the death of one patient at age 18 years and a heart-lung transplant in the other at age 8 1/2 years. Extensive gross pulmonary cysts were evident pathologically with diffuse panlobar emphysema microscopically. Studies of collagen and cultured fibroblasts in one patient revealed no abnormality. The early presentation, rapid progression and potentially lethal nature of lung involvement has not previously been emphasized.
The mothers of 72 children and adolescents with inflammatory bowel disease (IBD) and 44 mothers of children and adolescents with cystic fibrosis (CF) were given A-SADS interviews. Fifty-one percent of IBD mothers and 41% of cystic fibrosis mothers had a lifetime history of depression. More IBD than CF mothers had a history of suicide gestures or attempts, and were more likely to have a history of obsessive compulsive disorder. However, CF mothers were more likely to have experienced panic attacks.
The roles of insulin deficiency and insulin resistance in the pathogenesis of glucose intolerance in cystic fibrosis (CF) were evaluated in eight patients (aged 16.5 +/- 1.9 yr), four with normal glucose tolerance (NGT) and four with impaired glucose tolerance (IGT), and in seven healthy control (CN) subjects. First and second phase insulin secretions were evaluated during a hyperglycemic clamp. Hepatic glucose production (HGP) and insulin-stimulated glucose disposal were measured using [6,6-2H2]glucose and a stepwise hyperinsulinemic-euglycemic clamp. First and second phase insulin levels were significantly lower in both groups of CF patients compared with control values. There was an inverse relationship between glycohemoglobin level and first phase insulin (r = -0.81; P = 0.015) and second phase insulin (r = -0.97; P < 0.001). During the hyperglycemic clamp, the insulin sensitivity index was lower in CF-IGT, but not CF-NGT, compared with control values (6.66 +/- 1.79, 12.82 +/- 1.61, and 13.02 +/- 1.78 mumol/kg.min/pmol.L, respectively; P < 0.05). Basal HGP and fasting plasma glucose were higher in CF vs. CN [24.8 +/- 2.9 vs. 16.9 +/- 1.4 mumol/kg.min (P = 0.036) and 5.8 +/- 0.2 vs. 5.4 +/- 0.1 mmol/L (P = 0.035), respectively]. During the hyperinsulinemic euglycemic clamp, insulin-stimulated glucose disposal was significantly lower in CF-IGT (45.68 +/- 4.87 mumol/kg.min) vs. CF-NGT (78.99 +/- 1.34 mumol/kg.min) and CN (71.74 +/- 6.88 mumol/kg.min). Insulin sensitivity was lower in CF-IGT vs. CF-NGT (7.04 +/- 0.86 and 14.38 +/- 0.84 mumol/kg.min/pmol.L; P < 0.05). We conclude that 1) glycohemoglobin is a strong correlate of insulin deficiency in CF; and 2) glucose intolerance in this group of CF patients occurred as a consequence of concomitant insulin deficiency and insulin resistance.
Nine years after receiving a bone marrow transplant for aplastic anemia, a 14-year-old girl with severe pulmonary disease associated with graft-versus-host disease received a double lung transplant. Subsequent to lung transplant, her lung function improved dramatically (FEV1 increasing from 20 to 73 percent predicted normal, residual volume decreasing from 316 to 130 percent predicted normal values). The patient is currently well 15 months after transplant, while receiving immunosuppression consisting of FK506 and azathioprine. Double lung transplantation may offer a therapeutic option for the treatment of graft-versus-host pulmonary disease in selected patients.
For many years now percutaneous techniques have proved effective in the curative treatment of lumbar disc herniation, mostly in young subjects. This technique, however, is seldom indicated, let alone performed, in the cervical spine for a variety of reasons: a) the neck contains several closely arranged structures such as the vasculo-nervous bundles, the airway-digestive tract and the cervical spine around the spinal cord; b) the disc is approached by the anterior route, in contrast with the lumbo-sacral spine where the approach is posterolateral; c) the manufacturers insist on restrictions in the use of chymopapain in view of the potential risk of spinal cord damage, either by possible breaks in the meninges of by accidental diffusion of the enzyme into perimedullary epidural structures which support a particularly developed venous plexus; d) legal protection may be denied to operators who perform cervical chemonucleolysis, since the product has not yet been officially authorized, in France and perhaps elsewhere*, for treatment of cervical disc herniation. Several years of experience in the practice of cervical nucleolysis have convinced the authors that this method is remarkably effective and can be used in the treatment of cervicobrachial neuralgia (CBN) occurring in young subjects. Radiculalgia is essentially due to a disc fragment being positioned within the vertebral canal or a foramen, thereby compressing the nerve roots. During several years microsurgery of the disc has been effective in the treatment of refractory radiculalgia, and to compete with this procedure familiar to neurosurgeons cervical nucleolysis must convincingly demonstrate that its therapeutic value is at least as good as that of surgery. Finally, the vast majority of cervical disc herniations is made up of free disc fragments located within the meshes of the posterior longitudinal ligament of the spine (transligamentous fragment). Cervical nucleolysis was introduced in France by Bonafe and Lazorthes made enthusiastic by their first studies, and the idea of this procedure, developed by a radio-neurosurgical team, has gradually been gaining ground. The decision to intervene is made when the radiculalgia is recurrent or resistant to a well-conducted medical treatment of several weeks duration. Therefore all disc herniations that would have been treated surgically can be treated by injection of an enzyme. This point is very important, and in fact the well-codified and effective surgical procedure has progressively been abandoned by the operators, in view of the simplicity of nucleolysis and the rapid pain relief it procures.(ABSTRACT TRUNCATED AT 400 WORDS)
Previous studies have produced conflicting results about the effects of intracerebral injection of NGF after septal damage in rats: in one experiment, behavioral deficits in maze tasks were exacerbated by NGF administration whereas they were alleviated in another one. The present investigation aimed to clarify the effects of NGF and to identify factors liable to induce different behavioral outcomes. Behavioral effects were assessed following a postsurgical delay of five months using various parameters: food consumption in a novel environment, spontaneous activity, locomotion in an open-field, immobility in a tail suspension test, spontaneous alternation in a T-maze and performance in a radial eight-arm maze. Possible influence of intrahippocampal sympathetic fiber ingrowth occurring after septal lesions was ruled out, as the comparison of rats subjected to superior cervical ganglia removal with their lesion-control counterparts showed few behavioral differences, even after NGF administration. All lesioned rats showed reduced adaptability in most of these tests. Grafts partially reversed the lesion-induced deficit in spontaneous alternation. A single intracerebral NGF injection was found to ameliorate radial maze performance, whether rats were grafted or not. However, it appeared that the number of strategies available to NGF-rats in the radial maze task was as limited as for lesion-control rats. These findings suggest that NGF-rats do not recover spatial abilities lost after septal lesions, but are able to make more efficient use of remaining capacities to master the maze task.
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The Leyton Obsessional Inventory-Child Version was administered to 33 children with Crohn's disease, 11 with ulcerative colitis, and 46 with cystic fibrosis. Subjects with ulcerative colitis scored significantly higher on total number of obsessional symptoms and degree of resistance to symptoms than subjects with Crohn's disease. Scores of subjects with cystic fibrosis were intermediate between those of the ulcerative colitis and Crohn's disease groups. However, neither the ulcerative colitis group nor the Crohn's disease group differed significantly from the cystic fibrosis group that were statistically significant. Duration of illness was negatively correlated with symptoms in Crohn's disease and ulcerative colitis. The results suggest that obsessive-compulsive symptoms are not specific to Crohn's disease or ulcerative colitis and that obsessive-compulsive symptoms in pediatric chronic illness may be secondary to the demands of the illness.
The Quality of Well-being (QWB) Scale combines preference-weighted measures of symptoms and functioning to provide a numerical point in-time expression of well-being that ranges from zero (0) for death to 1.0 for asymptomatic optimum functioning. The QWB includes three scales of function: mobility, physical activity, and social activity. Each step of these scales is associated with preference weights. Preference adjustments for symptoms are also included. This paper describes how this general system was used to evaluate outcomes in three different clinical conditions: acquired immune deficiency syndrome (AIDS), cystic fibrosis, and arthritis. In one study, the QWB was administered to 31 patients participating in evaluation of azidothymidine (AZT) treatment for AIDS. The QWB system demonstrated substantial benefits of AZT treatment in comparison to placebo. In a second study, the QWB and a series of pulmonary function measures were administered to 44 patients with cystic fibrosis. The QWB was demonstrated to be significantly correlated with measures of pulmonary function, including FEV1 and maximal midexpiratory flow rate (MMEFR). In addition, there were significant associations between the QWB and measures of exercise tolerance. In the third study, the QWB and an arthritis-specific measure were administered to 83 arthritis patients before and after their treatment. The QWB was at least as capable of detecting clinical change in this population as was the disease-specific measure. For all three conditions, the QWB considered side effects and benefits of treatment in a common unit. Clinical trial data are cited to suggest that the QWB is a valuable outcome measure in arthritis treatment evaluation. We conclude that the QWB has substantial validity as a general health outcome measure and that the system can be used with different populations.
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To accurately assess the health knowledge, attitudes, and practices of students in grades four-seven, the staff of the School Health Education Evaluation (SHEE) project devoted extensive effort to identify a test appropriate for such assessment. An extensive literature review failed to produce an instrument sufficiently comprehensive or psychometrically sound that could be employed. In this paper, the philosophic orientation and the detailed processes followed in developing a noncurriculum specific, psychometrically sound evaluation instrument for use in the study is described. Included are the bases for the test framework, test objective development, test item review, the various measures constructed using the test blueprint, student performance on the test battery, the psychometric qualities of the test, and suggestions for appropriate use of the final test in other school health education settings.
Three patients with hemophilia presented with hemothoraces which resolved within 1-3 weeks following treatment with factor concentrates. Drainage of blood was not necessary. Eight previously reported cases are reviewed, and therapy and complications are summarized. We suggest that treatment of this rare complication of hemophilia with factor concentrates and avoidance of surgical intervention is indicated.
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The cases of two women who show postmenopausal bleeding and signs of endogenous estrogen production are presented. At laparotomy, ovarian hyperthecosis was found and confirmed histologically. Determination of 17 beta-estradiol concentrations in ovarian and peripheral veins suggested that these ovaries actively secreted excessive estrogens. Ovarian hyperthecosis is discussed as a cause of renewed endogenous estrogen activity in the postmenopausal women.