Showing papers by "Jewish Hospital published in 1998"

Autoregulatory Vasodilation of Parenchymal Vessels is Impaired during Cerebral Vasospasm

Abstract: Impaired CBF autoregulation during vasospasm after aneurysmal subarachnoid hemorrhage (SAH) could reflect impaired capacity of distal vessels to dilate in response to reduced local perfusion pressure or simply indicate that the perfusion pressure distal to large arteries in spasm is so low that vessels are already maximally dilated. Autoregulatory vasodilation can be detected in vivo as an increase in the parenchymal cerebral blood volume (CBV). Regional CBV, CBF, and oxygen extraction fraction in regions with and without angiographic vasospasm obtained from 29 positron emission tomography studies performed after intracranial aneurysm rupture were compared with data from 19 normal volunteers and five patients with carotid artery occlusion. Regional CBF was reduced compared to normal in regions from SAH patients with and without vasospasm as well as with ipsilateral carotid occlusion (P < .0001). Regional oxygen extraction fraction was higher during vasospasm and distal to carotid occlusion than both normal and SAH without vasospasm (P < .0001). Regional CBV was reduced compared to normal in regions with and without spasm, whereas it was increased ipsilateral to carotid occlusion (P < .0001). These findings of reduced parenchymal CBV during vasospasm under similar conditions of tissue hypoxia that produce increased CBV in patients with carotid occlusion provide evidence that parenchymal vessels distal to arteries with angiographic spasm after SAH do not show normal autoregulatory vasodilation.

Phenotypic expression of the DYT1 mutation: a family with writer's cramp of juvenile onset.

Abstract: Recently, the mutation causing early-onset generalized torsion dystonia has been identified as a GAG deletion in the gene for an adenosine triphosphate-binding protein named torsinA. We describe a German family with 5 clinically affected individuals carrying this mutation. In at least 4 of the 5 patients, the disease presented as a dystonic writer's cramp during late childhood or adolescence, which affected sequentially both sides but did not progress to a generalized form of dystonia. We conclude that familial writer's cramp may be a manifestation of the DYT1 mutation.

Long-term composite tissue allograft survival in a porcine model with cyclosporine/mycophenolate mofetil therapy.

Abstract: Background. Low-dose cyclosporine (CsA)'mycophenolate mofetil (MMF) therapy has significantly reduced the frequency of rejection and drug-induced side effects in rat hindlimb allograft recipients. With an eye toward direct clinical application, we developed a large-animal extremity composite tissue allograft model to assess the antirejection efficacy and systemic toxicity of combination CsA/MMF treatment. Methods. Radial forelimb osteomyocutaneous flap transplants were performed between size-matched, outbred pigs assigned to one of two groups: 5 control pigs received no immunosuppression, and 10 pigs received a once-daily oral CsA/MMF/prednisone regimen. Rejection was assessed by visual inspection of flap skin and correlated with serial histopathologic examination of skin biopsies. Results. In all control pigs, the flap was completely rejected on day 7. Of the 10 pigs receiving treatment, one died from pneumonia and an another from an anesthetic complication on days 19 and 30, respectively, without signs of rejection. Two flaps were lost on days 25 and 29 from severe rejection. Three pigs were free of rejection at the end of the 90-day follow-up period, and three had stable mild-to-moderate rejection at 90 days (P= 0.0007 vs. controls). White blood cell and platelet counts, serum creatinine values, and liver function tests remained normal in all animals receiving immunosuppressive therapy. Conclusions. Our results, to our knowledge, demonstrate for the first time that rejection can be significantly delayed in a large-animal composite tissue allograft model including skin using only orally administered agents dosed according to clinically relevant strategies without significant drug-specific systemic side effects.

Secondhand smoke, hypofibrinolysis, and Legg-Perthes disease

Abstract: In 39 children with Legg-Perthes disease who were nonsmokers, the specific aim was to assess relationships among parental cigarette smoking during pregnancy, household smoking before diagnosis of Legg-Perthes disease, hypofibrinolysis, and thrombophilia Fifteen (38%) children had no secondhand smoke exposure; 24 (62%) had secondhand smoke exposure before their diagnosis Seventeen (71%) of these 24 children were exposed while in utero to smoking by a parent or live in relative and also had exposure to household smoke during childhood; seven (29%) had only household smoke exposure in childhood In the full cohort of 39 children, secondhand smoke exposure correlated inversely with the major stimulator of fibrinolysis, stimulated tissue plasminogen activator activity Of the children exposed to smoking, 48% had low stimulated tissue plasminogen activator activity (< 219 IU/ml) compared with 7% of the children without secondhand smoke exposure and 14% of 22 healthy control children Secondhand smoke exposure had no significant effects on other measures of coagulation Secondhand smoke exposure while in utero and during childhood appears to lower stimulated tissue plasminogen activator activity and additionally may depress heritable low stimulated tissue plasminogen activator activity, leading to hypofibrinolysis Hypofibrinolysis may facilitate thrombotic venous occlusion in the head of the femur, leading to venous hypertension and hypoxic bone death, Legg-Perthes disease

Prospective assessment of coronary heart disease risk factors: the NHANES I epidemiologic follow-up study (NHEFS) 16-year follow-up.

Abstract: Objective: Our specific aim in the 16 year prospective NHANES I epidemiologic follow-up study (NHEFS) was to assess the important roles of modifiable dietary and behavioral factors in causation and prevention of deaths and hospitalizations for coronary heart disease (CHD).Methods: Using NHEFS 16 year follow-up data (1971 to 1987), we studied 5811 subjects, 1958 with and 3853 without CHD events, using logistic regression.Results: In age groups 40 to 49, 50 to 59, 60 to 69, and 70 to 74 years (at study entry in 1971–4), the numbers of men and women were respectively 597 and 1019, 570 and 619, 932 and 1042, and 486 and 546. The following factors were independently positively associated (p<.05) with CHD: age, serum cholesterol, body mass index, cigarette use, and region (Midwest, Northeast). The following factors were independently negatively associated (p<.05) with CHD: gender (female), race (black), fish intake, alcohol, high school education, moderate exercise, and moderate and heavy habitual physical acti...

Gastrointestinal symptoms and delayed gastric emptying in Fabry's disease: response to metoclopramide.

Abstract: The pathophysiology of gastrointestinal symptoms in patients with Fabry's disease is uncertain, despite the demonstration of histological and radiographic abnormalities of the gastrointestinal tract. The aims of this study were to determine if the gastrointestinal symptoms reported by patients with Fabry's disease are associated with abnormal gastric emptying and, if they are, whether prokinetic drug therapy would be beneficial. Ten patients with Fabry's disease had radionuclide gastric emptying studies performed to determine if gastrointestinal symptoms correlated with objective evidence of abnormal gastric emptying. A second study was performed in seven patients who received 5 days of therapy with oral metoclopramide. The mean percent solid gastric emptying for Fabry's patients was significantly less than that of a normal control group (40 vs 67%, P < 0.005). Five of seven patients with symptoms had abnormal gastric emptying. Six of seven symptomatic patients reported clinical improvement with metoclopramide. Of four symptomatic patients who had a repeat study after treatment, two showed improved emptying. In conclusion, our results suggest that gastrointestinal symptoms in Fabry's disease are frequently associated with delayed gastric emptying and that treatment with metoclopramide produces symptomatic and sometimes functional improvement.

A preliminary pilot study of treatment of thrombophilia and hypofibrinolysis and amelioration of the pain of osteonecrosis of the jaws

Abstract: Objectives. In a preliminary pilot study of 30 treatments in 26 patients with osteonecrosis of the jaws and chronic disabling facial pain, our specific aim was to determine whether, to what degree, and how safely therapy of hypofibrinolysis and thrombophilia would ameliorate the chronic pain associated with osteonecrosis of the mandible and maxilla. Study Design. Thrombophilia was treated with Coumadin (DuPont) in 10 patients; hypofibrinolysis was treated with Winstrol (Sanofi-Winthrop) in 20 patients, including 4 who had mixed thrombophilia and hypofibrinolysis and had previously been treated with Coumadin. The initial treatment period was targeted to be 4 months. Each patient was asked to keep a daily written pain-relief numeric rating score and side-effects diary and to provide a summary pain-relief numeric rating score and side effects compilation for the total treatment period. Results. There were 4 men and 22 women in the study group; their mean age was 49 ± 11 years. The mean onset of their osteonecrosis pain was at age 45 ± 12 years, and the mean duration of their facial pain prior to therapy was 4.5 ± 4.2 years. Ten patients had one or more thrombophilic traits (there were two patients with protein C deficiency, five with resistance to activated protein C and/or the mutant Factor V Leiden gene, and four with high anticardiolipin antibodies). The 10 patients who were thrombophilic were treated with Coumadin (the international normalized ratio was targeted to 2.5-3.0) for 22 ± 9 weeks. By self-reported pain-relief numeric rating scores, 6 of the 10 patients with thrombophilia (60%) had ≥ 40% pain relief, 2 (20%) had no change, and 2 (20%) had increased pain (30% and 80% worse). Nine of the 10 patients with thrombophilia (90%) had no Cumadin-related side effects; 1 patient (10%) stopped Coumadin therapy (after 28 weeks) because of nosebleeds. Winstrol (6 mg per day) was used for 16 ± 9 weeks in 20 patients with hypofibrinolysis, some of whom had one or more hypofibrinolytic traits (10 had high levels of plasminogen activator/inhibitor activity, usually accompanied by low stimulated tissue plasminogen activator activity; 13 had high Lp[a] lipoprotein). Of these 20 patients with hypofibrinolysis, 9 patients (45%) had ≥40% pain relief, 3 patients (15%) had 20% to 30% relief, 5 patients (25%) had no improvement, and 3 patients (15%) had increased pain (30% worse, 60% worse, and 70% worse). Six of the 20 patients with hypofibrinolysis (30%) had no Winstrol-related side effects, while 14 (70%) had side effects that could be attributed to Winstrol, including weight gain, peripheral edema, increased facial and body hair, and acne—all of which were reversed within 6 weeks of stopping Winstrol therapy. Conclusions. We postulate that thrombophilia and hypofibrinolysis lead to impaired venous circulation and venous hypertension of the mandible/maxilla with subsequent development of osteonecrosis and chronic facial pain. In many patients, facial pain can be ameliorated by treating the pathogenetic coagulation defects with Coumadin or Winstrol. Large, double-blind, placebo-controlled crossover studies will be required in the future to validate these preliminary results and to determine whether pain relief with Coumadin or Winstrol justifies the risks and side effects associated with these medications, especially for long-term use, in osteonecrosis of the jaws.

Müllerian Inhibiting Substance Improves Longevity of Motility and Viability of Fresh and Cryopreserved Sperm

Abstract: Mullerian inhibiting substance (MIS) is a hormone present in seminal plasma, but its role is unknown. In this study, the effects of MIS on sperm survival in fresh and cryopreserved specimens were investigated. Fresh sperm motility and viability (n = 12) were evaluated in specimens after 0, 0.5, 1, 3, 5, and 22 hours of incubation in the presence or absence of MIS. Motile and nonmotile sperm were evaluated in Cell-Vu counting microscope slides, and viability was assessed by eosin-nigrosin exclusion. Sperm cryopreserved for 2 weeks in TES (N-Tris[hydroxymethyl]methyl-2-aminoethanesulfonic acid)-Tris-glycerol-egg yolk buffer and 4% glycerol with or without MIS were thawed at room temperature (n = 6) and were evaluated for motility and viability using identical methods to those used with fresh sperm. The effects of MIS were examined by coincubation with monoclonal anti-MIS antibody (6E11; n = 6). In fresh and cryopreserved sperm incubated with MIS, both motility and viability were higher than in the absence of MIS (P < 0.03; Wilcoxon signed rank test) at 5 and 22 hours. Coincubation with anti-MIS antibody eliminated the effects of MIS. Longevity of sperm motility and viability are improved both in fresh and cryopreserved sperm in the presence of MIS and may have potential for use in assisted reproductive technology.

Laser wire for crossing chronic total occlusions : Learning phase results from the U.S. TOTAL Trial

Abstract: The Prima laser guidewire system (Spectranectics Corp., Colorado Springs, CO) consists of an 0.018" hypotube containing a bundle of 45-microm optical fibers coupled to a pulsed excimer laser operating at a tip fluence of 60 ml/mm2 and a repetition rate ranging from 25-40 Hz. This laser guidewire was specifically designed to cross total occlusions refractory to passage with conventional wires. The Prima wire was evaluated in a feasibility study at 15 U.S. centers. Following failure to cross a total occlusion with approved guidewires, the Prima wire was utilized in 179 patients. Average age of subjects was 61 yr. Lesion locations included left anterior descending (36%), right (45%), and circumflex (19%) coronary arteries. Mean angiographic age of total occlusions was 70 wk (range, 2-1,020 wk, median, 14 wk). The use of the Prima wire either solely or in combination with conventional guidewires resulted in successful crossing in 61% of these previously impenetrable occlusions. Failure of the device was commonly related to length of the occlusion and tortuosity along the occluded pathway. Major complications included myocardial infarction in 7 patients (3.9%), tamponade in 3 (1.7%), and death in 2 (1.1%). This "learning phase" pilot study confirmed the feasibility of a laser guidewire in chronic total occlusions that are resistant to passage of conventional guidewires. An extended registry at these investigative sites is planned.

Parents' Perceptions of Their Children's Lung Transplant Experiences:

Abstract: There is little informationfrom the perspective of parents living with a child who has undergone lung transplantation. A phenomenological approach integrating Schutz's postulatesfor the construction of social reality with Colaizzi's method of analysis was used to investigate the meaning of the experience. Fifteen parents of 12 children were interviewed. Theme clusters reflecting the meaning of the stages of the transplantation experience were formulated. Parents perceived lung transplantation as creating hope for the future, creating a context of uncertainty and an opportunity for normalcy, and promoting the development of a new perspective on life. Humanistic nursing practice theory provided the link between study results and nursing practice. Implicationsfor nursing practice were identified.

MR imaging of the shoulder after an impingement test: how long to wait.

Abstract: OBJECTIVE: The purpose of this study was to determine the appropriate minimum waiting time between an impingement test with subacromial injection and subsequent MR imaging to avoid misinterpretation if the injected fluid is still present. CONCLUSION: MR imaging should be delayed a minimum of 24 hr after a subacromial injection. Fluid in the subacromial space 24 hr after subacromial injection is unrelated to an impingement test.

Reimbursement and documentation issues in an ambulatory ECT program.

Abstract: In this era of decreased health resources and the strong influence of third-party payers, the financial advantages of ambulatory electroconvulsive therapy (ECT) should be well recognized by all concerned parties. However, to a large degree, third-party payers are only minimally aware of the availability and potential cost savings associated with this treatment modality. Also, documentation has been problematic, because reviewers unfamiliar with ECT have not been able to find the necessary data even when they are present in the chart. This article illustrates some of these problems and how they have affected our ambulatory ECT program.

Machiavellianism and tolerance of ambiguity

Abstract: The relationship of Machiavellianism and Tolerance of Ambiguity was studied in 47 African-American college and university administrators. The mean score on Machiavellianism in the youngest group (30–40 yr.) of administrators was significantly higher on Machiavellianism than the mean of the oldest group (50 yr. and older) of administrators.

Collaborative end-of-life decision making in end stage renal disease.

Abstract: Patients newly diagnosed with end stage renal disease are faced with many treatment decisions. The decision-making process should follow some well-defined steps. These start with the decision to initiate dialysis and may end with a 'do not resuscitate' request or even a withdrawal from dialysis. The nephrology team can lead the patient and family through this process by providing timely, realistic information to help them make the best decisions. End-of-life decision making should be a part of the initial long-term care planning done with every patient and family.

Pediatric Oncology in Brazil

Abstract: Brazil is a country with many contrasts. This can help the understanding of environmental factors influencing the incidence of different cancers. The standard treatment and results achieved in certain areas of the country are not encountered in others. The establishment of national cooperative groups has helped the improvement. The subspecialty has been recognized and training programs are available. Many scientific achievements have been developed and are described.

Postmortem diagnosis of leukodystrophies

Abstract: Leukodystrophies are progressive disorders involving the development and maintenance of myelin in the central and peripheral nervous systems. Although relatively uncommon, leukodystrophic disorders may be undiagnosed or misdiagnosed during life, and may appear as "sudden death." In such instances, these victims may be referred to a forensic pathologist. In general, leukodystrophies are inherited in an autosomal recessive manner so that proper postmortem diagnosis by the forensic pathologist is extremely important to the decendant's family for future family planning.

Nesidioblastosis: a case study.

Abstract: Hypoglycemia is a frequent problem in the neonatal period requiring close attention and intervention. Severe, persistent hypoglycemia can have various etiologies; one of the most common causes is hyperinsulinism. Nesidioblastosis, although rare, is the most common cause of hyperinsulinism in the neonate. If not detected early, nesidioblastosis can lead to brain damage and death secondary to severe hypoglycemia. The etiology of nesidioblastosis remains unclear. Treatment involves maintaining normal blood glucose levels; treatment modalities include high glucose infusion rates, use of medications, and surgical intervention. The article reviews the pathophysiology and treatment modalities for nesidioblastosis. A case study is also presented that describes the clinical presentation, treatment, surgical intervention, and postdischarge clinical course of an infant with nesidioblastosis.

Anesthetic management of Takayasu's arteritis for extra-anatomic thoraco-abdominal aorto-aortic bypass: a case report.

Abstract: Takayasu's arteritis, also known as Takayasu's syndrome, is a chronic inflammatory disease, which primarily affects large vessels including the aorta and its main branches. The anesthetic management for the patient presenting with Takayasu's arteritis is complicated by the multiple organ systems affected by the disease. A 59-year-old Chinese female with the diagnosis of Takayasu's arteritis presented with severe progressive occlusion of the thoracic and abdominal aorta and bilateral renal artery stenosis, necessitating an extra-anatomic aorto-aortic bypass and bilateral aorto-renal bypass. The patient tolerated the operative procedure without any untoward events. This case report discusses the pathophysiology of Takayasu's arteritis and the anesthetic management of aortic surgery specific to this particular disease process.