显微神经外科手术治疗小脑血管网状细胞瘤32例

1临床资料 2000-03／2003—12共收治小脑血管网状细胞瘤患者32（男21,女11）例,年龄24～59岁,平均38岁．病程1．6～6a,平均1．2a,其中病程1a内共23例,占70％．按类型分为囊肿结节型30例,实质肿块型2例．本组有家族史2例．表现有颅内压增高征27例,共济失调20例,眼球震颤15例,脑神经受损7例．均于术前行颅脑CT和MRI平扫加强化扫描检查,囊肿直径2．5～5．5cm．     

Pindborg tumor in children

Pindborg tumor or calcifying epithelial odontogenic tumor is a rare benign neoplasm. The average age at diagnosis is 40 years without a significant prevalence for one sex. The most frequent localization is the mandibular premolar and molar area; less frequently the lesion is found in the maxilla, while other localizations like the maxillary sinus are extremely rare. Ethiology of this lesion is not clear. The complete surgical removal of the lesion is usually considered the most common type of treatment. The authors report a peculiar case of Pindborg tumor characterized by a rare localization of the lesion (maxillary incisal area) and by the young age of the patient (nine years old). The correct treatment in the case of a small mass is the surgical enucleation of the lesion. The purpose of the surgical treatment is the radical resection of the neoplasm with clean margins in order to reduce the risk of recurrence of disease. Recurrence of disease many years after surgical therapy has been reported that is why a clinical and instrumental long-term follow-up of the patient are suggested.     

Arginine vasopressin does not alter mucosal tissue oxygen tension and oxygen supply in an acute endotoxemic pig model

Objective To determine the effects of increasing dosages of continuously infused arginine-vasopressin (AVP) on mucosal tissue oxygen tension and oxygen supply in an auto-perfused, innervated jejunal segment in an acute endotoxic porcine model.Design Prospective, randomized, experimental study.Setting University hospital animal research laboratory.Interventions Jejunal mucosal tissue PO₂ was measured employing two Clark-type surface oxygen electrodes. Oxygen saturation of jejunal microvascular hemoglobin was determined by tissue reflectance spectrophotometry. Systemic hemodynamic variables, mesenteric-venous and systemic acid base and blood gas variables and lactate measurements were recorded. Measurements were performed at baseline, after E. coli lipopolysaccharide (LPS) administration and at 20 min intervals during incremental AVP infusion ( n =8; 0.014, 0.029, 0.057, 0.114 and 0.229 IU kg^-1 h^-1, respectively) or infusion of saline ( n =8).Measurements and results LPS infusion leads to a significant ( P <0.05) decrease of mucosal tissue oxygen tension (PO₂muc, 24±3 to 12±2 mmHg) and microvascular hemoglobin oxygen saturation (HbO₂, 38±4 to 21±4%). Mesenteric venous lactate level increased (2.4±0.3 to 4.7±1.7 mmol l^-1), while mesenteric venous pH decreased (7.38±0.02 to 7.26±0.12), indicating tissue hypoxia. AVP significantly increased mean arterial pressure (MAP, 81±15 to 97±17 at 0.057 IU kg^-1 h^-1). No differences in jejunal mucosal oxygenation occurred between study groups at any dosage during the experimental protocol.Conclusion AVP administration did not further compromise mucosal tissue oxygen tension and oxygen supply in the acute phase of endotoxic pigs.This article refers to the editorial     

Ophthalmological manifestations in segmental neurofibromatosis type 1

AIMS: To study the ophthalmological manifestations in individuals with the typical features of neurofibromatosis type 1 (NF1) circumscribed to one or more body segments, usually referred to as segmental NF1. METHODS: Visual acuity and colour tests, visual field examination, slit lamp biomicroscopy of the anterior segment, and a detailed examination of the retina by indirect ophthalmoscopy were performed at diagnosis and follow up in 72 consecutive subjects (29 males, 43 females; aged 1-64 years; mean age 14.6 years) seen at the university departments of paediatrics in Catania and Rome, Italy, during years 1990-2003, who had in restricted body areas: (1) typical pigmentary manifestations of NF1 (cafe au lait spots and freckling) only (n = 48); (2) NF1 pigmentary manifestations and neurofibromas alone (n = 2); (3) neurofibromas only (n = 15); and (4) plexiform neurofibromas only (n = 7). RESULTS: None of the 72 patients had Lisch nodules in the iris irrespective of age at eye examination or hypertelorism (a "minor" NF1 feature) and none developed typical associated ophthalmological NF1 complications. An additional child had an isolated optic pathways glioma (OPG), which behaved both biologically and radiographically as an NF1 associated OPG. CONCLUSIONS: This represents the first systematic study reporting on eye involvement in the largest series of individuals at different ages having segmental NF1. As one of the postulated mechanisms to explain segmental NF1 is somatic mosaicism for the NF1 gene (so far demonstrated only in two patients) the present findings could be explained either by the fact that the eye is too far from the mutated area with NF1 lesions in most cases or by the NF1 (or other "predisposing" or "cooperating") gene mutation restricted to too few cellular clones or to tissues embryologically different from the eye.     

Craniosynostosis: diagnostic value of three-dimensional CT reconstruction

Three-dimensional computed tomography (CT) has an important role in determining the presence and extent of congenital and acquired craniofacial deformities. The authors compared the sensitivity and specificity of three-dimensional CT in the detection and characterization of craniosynostosis with that of planar CT and skull radiography. Eighty-two patients with isolated and syndromal synostoses were imaged with CT and three-dimensional CT, and 42 with skull radiography. Three-dimensional CT scan processing was performed by shaded-surface reconstruction, volumetric, and depth-coded methods. Two trained observers read each scan series in a blinded fashion. Diagnostic utility of the images was determined with receiver operating characteristic analysis. The observers ranked three-dimensional shaded images higher than the other types, with three-dimensional volumetric images second and three-dimensional surface images ranked third. Results of this study demonstrate that three-dimensional shaded-surface reconstruction from CT scans is superior to conventional plain radiographs and CT scans in diagnosing craniosynostosis.     

Surgical strategies in the treatment of symptomatic osteomas of the orbital walls.

We present a series of 19 surgically treated osteomas involving the orbital walls. Eight were localized at the junction of the frontal sinus and orbital roof, three were frontoethmoidal, two were of the orbital roof, one of the lateral wall, one of the orbital floor, and four involved all the walls of the orbit and were termed panorbital. All tumors at the junction of the frontal sinus and the orbital roof as well as those originating from the orbital roof proper were removed by frontal craniotomy. In the three that were frontoethmoidal, osteotomies were performed. Lateral and inferior orbitotomies were done when the corresponding orbital walls were affected. The four panorbital osteomas required large frontotemporal craniotomies in association with superolateral orbitotomy and zygomatic and/or malar osteotomy, when necessary. Satisfactory cosmetic and functional results were obtained in all but two cases of panorbital osteomas, in which the neurological deficit worsened after surgery. We discuss the choice of the various approaches described, with reference to the relevant literature.     

人参的分析——Ⅳ.人参皂甙的高效液相色谱测定

本文报道了人参中六种主要皂甙(Rb_1,Rb_2,Rc,Rd,Re,Rg)的HPLC测定法,用氨基键合相柱,示差折光检测,流动相为甲醇中乙腈—乙二醇—醋酸铵(0.14 mol/L)(30:70:5:10.6),同时还改进了纯化方法,此法快速、重现性好,与薄层比色法比较结果基本一致。