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1/262. The value of intravenous heme-albumin and plasmapheresis in reducing postoperative complications of orthotopic liver transplantation for erythropoietic protoporphyria.

    Erythropoietic protoporphyria (EPP) is marked by a deficiency of ferrochelatase, which occurs in all cells and tissues, preventing effective conversion of proto porphyrin IX to heme and thereby blocking effective feedback inhibition of heme synthesis. The major source of the excess protoporphyrin is the bone marrow. Protoporphyrin IX may accumulate, with resultant toxicity chiefly of the marrow, skin, nervous system, and liver. Orthotopic liver transplantation (OLT) is, at present, the only adequate intervention for severe liver compromise secondary to protoporphyrin deposition, but it has been complicated by severe photosensitivity and polyneuropathy. Intravenous heme and plasmapheresis have been proposed but not previously reported as means to reduce the protoporphyrin burden before liver transplantation. We report a man with EPP who underwent preoperative heme-albumin administration and plasmaphereses that led to marked reductions in plasma and erythrocyte protoporphyrin levels. His OLT was uneventful, and he developed neither polyneuropathy nor exacerbation of photosensitivity.
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keywords = nervous system
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2/262. classification and treatment of intercondyloid fractures of the humerus.

    The authors describe the satisfactory results obtained in sixteen intercondyloid fractures of the humerus, thirteen of which were treated surgically. The preference for surgical treatment in such fractures is based in the assumption that, as in all articular fractures, a good functional result can only be achieved if there is the most perfect possible reconstruction of the fragments and the joint surface. A classification is therefore suggested which is based not purely on anatomical criteria, but is also related to treatment and prognosis. The slendor nature of the distal end of the humerus and the danger of metal reaction call for the use of fixation devices that are efficient but slender, such as fine screws and crossed wires. The precise method of fixation is conditioned above all by the direction of the fracture lines. More solid fixation with early mobilisation can be achieved by compression screws, and less solid fixation with longer immobilisation is achieved by fixation with crossed Kirschner wires. Consequently, the more oblique types of fracture with fragments with long beaks that allow more stable fixation with compression screws have the more favourable prognosis. In the evaluation of results, the authors emphasize the importance of using parameters which take into account the functionally useful range of joint movement.
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ranking = 1.2733486953747
keywords = metal
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3/262. acinetobacter meningitis: four nosocomial cases.

    We report the clinical features and therapeutic outcomes of four patients with multiantibiotic-resistant acinetobacter meningitis. There were three males and one female, aged from 17 to 49 years. Three of them had suffered from head injuries with skull fractures, and the other suffered from an intracerebral hemorrhage and underwent a craniotomy. All four patients acquired nosocomial acinetobacter meningitis, and multiantibiotic resistance developed. After treatment with imipenem/cilastatin, three of the four patients survived; one died of multiorgan failure. Because the clinical manifestations of acinetobacter meningitis are similar to those of other gram-negative bacillary meningitis, the diagnosis can only be confirmed by bacterial culture. Resistance to multiple antibiotics, including third-generation cephalosporins, is frequently seen in patients with nosocomial acinetobacter meningitis, and imipenem/cilastatin seems to be the antibiotic of choice for this potentially fatal central nervous system infection.
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keywords = nervous system
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4/262. Recurrent Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: report of a patient with histologically similar but clonally distinct metachronous abdominal and brain lesions.

    A liver transplant patient developed a single central nervous system (CNS) intraparenchymal lesion 5 months after the diagnosis of an intraabdominal diffuse large B-cell post-transplant lymphoproliferative disorder (PTLD). biopsy of the new CNS lesion showed a diffuse large B-cell PTLD morphologically and immunohistochemically indistinguishable from the abdominal lesion. In addition, both lesions were positive for Epstein-Barr virus (EBV) dna by polymerase chain reaction (PCR) and for EBV-encoded rna by in situ hybridization. Although these results were consistent with a metastatic origin for the CNS lesion, the finding of an intraparenchymal lesion without leptomeningeal or dural spread was suggestive of a new primary CNS lymphoma. Proof that the brain lesion was a second primary and not a metastasis was obtained by immunoglobulin gene rearrangement studies and assessment of EBV clonality. Multiple primary lymphoid neoplasms arise at higher frequency in the setting of immunosuppression, and molecular investigations of tumor clonality can provide clinically relevant staging and prognostic information.
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5/262. Postoperative airway stenosis and stent therapy in carinal reconstruction for lung cancer.

    A 48-year-old male had adenocarcinoma of the right upper lung lobe that invaded the lower trachea. The right upper lobe, the carina, and 5 rings of the lower trachea were resected. The carina was reconstructed using end-to-end anastomosis between the trachea and right intermediate bronchus, with the left main bronchus anastomosed to the side wall of the intermediate bronchus. Two months after surgery, the right intermediate bronchus developed bronchomalacia and the tracheal anastomosis granulatory stenosis. bronchomalacia was treated with 2 expandable metallic stents, and granulatory stenosis with a Dumon stent. Although the silicone stent successfully dilated the granulatory stenosis, the metallic stents caused delayed glanulatory stenosis. We concluded that a metallic stent is not desirable for treating postreconstructive airway stenosis including bronchomalacia, whereas a Dumon stent may be effective.
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ranking = 3.8200460861241
keywords = metal
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6/262. Stereotactic radiosurgery. VIII. The classification of postradiation reactions.

    Postradiation reactions in the CNS are well described and catalogued in the conventional radiotherapy literature; acute, subacute and late CNS reactions are recognized. Tumours predispose to these normal tissue reactions by the oedema and pressure epiphenomena that occur in their environs, and probably by other mechanisms associated with tissue breakdown. That late normal tissue reactions (particularly permanent late sequelae--universally referred to as 'necrosis') occur in the normal nervous system is in the complication list of every radiosurgery centre. This article, for the first time, places postradiosurgery observations within or without the existing classification of reactions, and draws attention to the fact that previous 'radiosurgery risk factor' papers in the literature may be wrong to pool different 'reactions' in the formulation of risk formulae for normal brain damage following single shot radiotherapy. Acute reactions occur in the same manner as described for conventional radiotherapy, being a transient swelling phenomenon that occurs 12-48 h after therapy; they are fully reversible and do not usually augur late problems; routine administration of short duration steroids around the time of radiosurgery may prevent or delay the clinical signs. Subacute reactions occur 3-10 months later (a later time than the subacute reactions following conventionally fractionated radiotherapy), and may prove fully or partially reversible, or progress to permanent sequelae; the difference between these and late sequelae (which tend to be permanent themselves) then becomes blurred. That tumour swelling occurs in the subacute phase and is associated with oedema in the surrounding normal brain is an interesting observation (occurring in extra- and intra axial slow-growing tumours); it denotes tumour damage and has not been encountered in the conventionally-fractionated radiotherapy literature. Tumour shrinkage occurs later, with subsidence of the surrounding oedema, and this phenomenon may therefore be regarded (paradoxically) as a good prognostic sign, a point about which the referring clinician should be made aware. Similarly, contrast enhancement in the tumour perimeter at this time reflects a host reactive response and not tumour activity. Persistent clinical neurological signs and MRI changes (best seen on the T2 weighted sequences) beyond 2 years, indicate late damage or reaction. Usually, this represents scarring or coagulative necrosis without mass effect, but if there is a low signal area with mass effect and considerable surrounding oedema, liquefactive necrosis has occurred and (as in the brachytherapy literature) surgical decompression is very occasionally needed.
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keywords = nervous system
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7/262. trimethoprim-sulfamethoxazole-induced tremor in an immunocompetent patients.

    trimethoprim-sulfamethoxazole (TMP-SMX) is a widely administered antibiotic that is well tolerated by most patients. hypersensitivity reactions and gastrointestinal intolerance are the most common adverse events associated with it. central nervous system adverse effects such as tremors are less common and occur primarily in patients with acquired immune deficiency syndrome. A 29-year-old immunocompetent man developed a tremor while taking TMP-SMX. The tremor resolved within 2 days after the drug was discontinued.
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keywords = nervous system
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8/262. magnetic resonance imaging artifact following acoustic neurofibroma surgery--case report.

    Metallic artifacts in magnetic resonance (MR) imaging occur mostly in patients who have received an implant at surgery. Similar artifacts are now increasingly recognized in patients in whom high-speed drills have been used. A 15-year-old male with neurofibromatosis 2 had undergone excision of acoustic neurofibroma on the left 1.5 years prior to the present admission. MR imaging to evaluate the acoustic neurofibroma on the right showed a metallic artifact at the site of the previous surgery. Computed tomography did not show any evidence of metal debris. The artifact was probably caused by metallic dust or debris from a high-speed drill during the first surgery. We suggest that care should be taken to prevent deposition of such debris in the operative field to prevent this complication.
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ranking = 3.8200460861241
keywords = metal
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9/262. Spontaneous epidural hematoma following a shunt in an infant with congenital factor x deficiency. Case report and literature review.

    The authors describe a case of an infant with congenital factor x deficiency. The patient presented with a central nervous system hemorrhage followed by hydrocephalus. He underwent a ventriculoperitoneal shunt and, during the postoperative period, developed a spontaneous epidural hematoma, which was evacuated. The clinical and pathophysiological aspects of this case are discussed based on a literature review.
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ranking = 1
keywords = nervous system
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10/262. propofol for sedation and control of intracranial pressure in children.

    Following central nervous system insults, control of intracranial pressure may lessen the incidence of morbidity and mortality. Therapies to control intracranial pressure include osmolar agents, prevention of and control of seizures, drainage of cerebrospinal fluid, hypothermia, and barbiturates. Control of agitation and excessive patient movement are additional components in the management of ICP. Although opioids and benzodiazepines are generally effective, in a small subset of patients, alternative agents may be necessary. The authors present 2 children with increased ICP in whom propofol was used to provide sedation and control ICP. The use of propofol in this setting and its possible applications in the children with increased ICP are discussed.
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keywords = nervous system
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