50例城市巨幼细胞性贫血病因分析

目的：了解城市中营养性巨幼细胞性贫血的常见病因。方法：通过回顾性分析病史及有关辅助检查结果，了解本院收治的50例营养性巨幼细胞性贫血患者的发病原因。结果：17例患者因患胃肠病等器质性疾病而发病，部分病例有多种病因同时作用；29例患者因单纯的膳食习惯异常发病；4例患者未发现明确病因。引起膳食习惯异常的原因有口腔疾患，以及各种生物、社会精神因素引起的不良生活方式。结论：除各种疾病外，不良生活方式引起的膳食习惯异常也是现代城市中营养性巨幼细胞性贫血的常见病因，应引起临床及公共卫生专家重视。     

巨幼细胞性贫血74例临床分析

目的 分析74例巨幼细胞性贫血病因、临床表现、外周血及骨髓象变化。方法 对74例经骨髓象检查的巨幼细胞性贫血患者的临床及化验结果进行回顾性分析。结果 74例中40例（54．05％）有消化道症状，38例（51．35％）三系血细胞均减少，12例（16．22％）有神经症状，巨幼细胞性贫血需与全血细胞减少的疾病和消化道疾病及神经精神疾病进行鉴别。结论 对于临床表现不典型且血细胞异常的患者，应积极动员患者尽早做骨髓穿刺检查，注意寻找多分叶核的粒细胞及巨型杆状核细胞的存在，及时明确诊断，注重消化系统疾病的防治及膳食结构调整，维生素B12和（或）叶酸治疗，绝大部分患者治疗效果显著。     

巨幼细胞性贫血患者临床及实验分析

目的:分析巨幼细胞性贫血(MA)的病因、发病年龄、黄疸程度、血清乳酸脱氢酶(LDH)水平、贫血程度及其与白细胞和血小板三系列之间的关系。方法:对92例经骨髓象检查确诊的MA患者同时检测血液常规、胆红素及LDH,并对临床及实验资料作回顾性分析。结果:消化系统器质性病变70例,膳食异常47例,部分患者多种病因并存,尤其甲状腺功能减退症有6例;患者平均年龄(59.35±17.74)岁,女55例,男37例,红细胞(1.54±0.52)×1012/L,血红蛋白(61.87±16.86)g/L,三系列减少占0.54,中、重度贫血86例,总胆红素(29.79±21.81)mmol/L,LDH(987.32±789.36)U/L。结论:消化系统器质性病变及膳食异常是引发MA的主要原因,而甲状腺功能减退症亦可致MA,发病以中、老年人居多,女性多于男性,贫血多为中、重度,半数以上患者周围血可表现三系列减少,血清LDH水平明显增高并表现一定程度的黄疸,且黄疸与LDH正相关。     

苯妥英钠致巨幼细胞性贫血一例

１　病例介绍患者,女,２４岁。反复腹泻伴头晕、乏力、食欲不振１年余,加重１个月于１９９７年８月１５日入院。患者自１９８８年诊为“癫痫”后一直服用“苯妥英钠”治疗。查体：体温３７．２℃,重度贫血貌,表情淡漠,皮肤粘膜苍白,无黄染,无出血点,舌乳头萎缩,肝脾不大,神经系统检查无异常发现。实验室检查：ＷＢＣ３．７×１０９／Ｌ,Ｎ０．４９,Ｈｂ４６ｇ／Ｌ,ＭＣＶ１１４．３ｆｌ,ＰＬＴ１８３×１０９／Ｌ。骨髓象：增生极度活跃,Ｇ∶Ｅ＝０．７５∶１,红系及粒系均有部分细胞呈巨幼变,分叶核可见多分叶现象,红系…     

38例老年人巨幼细胞性贫血临床分析

巨幼细胞性贫血（megaloblastic anemia，MBA）是由于叶酸或维生素B12缺乏导致DNA合成障碍的大细胞性贫血，多见于学龄前儿童、妊娠和哺乳期妇女。但近年来老年人巨幼细胞性贫血有增高的趋势，为探讨老年人MBA的病因、发病规律及临床特征，对我院1999年6月至2007年6月收治的38例老年人MBA患者的病因、临床特征等进行回顾性分析，现报告如下。     

难治性贫血与巨幼细胞性贫血的临床特点分析

难治性贫血与巨幼细胞性贫血是两种不同的疾病，难治性贫血常以红细胞减少、出血、贫血、感染、骨髓中出现红系、粒系、巨核细胞质和量的改变。巨幼细胞性贫血是以叶酸、维生素B12缺乏引起细胞内脱氧核糖核酸(DNA)合成障碍，导致细胞的巨型改变。但两者在临床、实验室检查、骨髓形态学有相似之处，容易混淆，甚至误诊。本文对我院血液病研究室近年来确诊的难治性贫血18例，巨幼细胞性贫血76例，进行临床及形态学的观察。现报告如下。     

老年人巨幼细胞性贫血36例临床分析

目的探讨老年人巨幼细胞性贫血的临床特点。方法对36例老年人巨幼细胞性贫血住院患者的资料进行分析。结果老年人巨幼细胞性贫血男性多见,男女比例为21,伴有心、脑血管疾病等内科其他疾病29例,占81％;以非血液病诊断入院的20例,占56％;外周全血细胞减少22例,占61％。骨髓有核细胞增生程度均在活跃以上。血清乳酸脱氢酶升高的32例,占89％。维生素B12减少者20例。检出伴有慢性消化道疾病者(慢性胃炎,胃、十二指肠球部溃疡,胃息肉,十二指肠憩室,食道憩室,结肠憩室,食道裂孔疝,慢性结肠炎,胆囊炎、胆石症)34例,占94％,贲门癌1例,占2.8％。用叶酸、维生素B12治疗效果显著。结论老年人巨幼细胞性贫血常因非血液病就诊而易于漏诊。临床上应与再生障碍性贫血、骨髓增生异常综合征(MDS)相鉴别。老年人巨幼细胞性贫血的发生与存在的消化道疾病关系密切,进行相关检查可避免遗漏潜在的疾病。     

巨幼细胞性贫血30例临床分析

为了及时正确的诊断巨幼细胞性贫血，避免误诊，误治，对３０例巨幼贫患者进行分析。认为巨幼贫多见于妊娠期妇女和婴幼儿，但近年来老年人发病有明显的增多趋势。本组２０例患者均有不同的慢性消化道疾病，提示巨幼贫与消化道病变关系密切，巨幼贫可并有溶血现象和出血倾向。巨幼贫诊断明确后治疗效果显著，预后好。     

巨幼细胞性贫血患者骨髓检查结果分析

目的：了解巨幼细胞性贫血患者骨髓及实验室检查结果。方法：对222例巨幼细胞性贫血患者的骨髓象及血液的红细胞、白细胞、血小板和血清叶酸、维生素B12等结果进行总结分析。结果：有129例（58.1%）表现为全血细胞减少,以叶酸缺乏为主185例（83.3%）。叶酸、维生素B12缺乏越严重,骨髓中巨变等病态造血越明显。结论：巨幼细胞性贫血患者骨髓造血病态的程度往往反映患者营养不良的程度。     

巨幼细胞性贫血伴环铁粒幼细胞增多误诊为骨髓增生异常综合征的临床分析

目的通过对巨幼细胞性贫血（MA）同时伴环铁粒幼细胞增多和骨髓增生异常综合征（MDS）的骨髓象的分析,进一步提高MA和MDS的诊断和鉴别诊断水平。方法结合临床资料对我院2009年1月～2011年1月诊治的5例MA患者进行外周血、骨髓细胞形态学分析。结果 5例MA患者外周血检查均为3系减少,MCV值〉100 fl;骨髓象检查巨幼红细胞增多,全血细胞系呈现核畸形等类病态造血象;骨髓铁染色见内外铁明显增多,可见环形铁粒幼细胞,初诊结果支持MDS。但均未见红细胞系奇书核、粒细胞间桥、小淋巴样巨核细胞等典型诊断MDS的较明确的病态造血形态学标志,故考虑为MA。给予叶酸、维生素B12治疗后临床症状明显改善,支持MA的诊断。结论提高对血液病异常造血的认识,反复推敲仔细观察细胞形态,结合病史、染色体检查、组化检查,可进一步提高MA和MDS的诊断和鉴别诊断水平。     

视觉诱发电位在视神经病变中的诊断价值

采用四种不同空间频率的图形视觉诱发电位（VEP）对视神经病变（包括外伤性视神经病变、视神经炎、缺血性视神经病变、视神经萎缩）患者进行检查,并与正常对照组比较。结果显示：①视神经病变组P100波振幅、潜伏期改变有非常显著性差异（P＜0．05）。②视神经病变组内各疾病的P100波振幅改变无显著差异（P＞0．05）,而视神经炎患者的潜伏期明显延长（P＜0．05）。③视神经病变组的病程与P100波潜伏期呈正相关,与振幅无关。④视神经病变组的视力与P100波振呈正相关。提示VEP对视神经病变诊断有重要价值,采用多种空间频率刺激可增加VEP的敏感性。     

Impaired visual evoked potential and primary axonopathy of the optic nerve in the diabetic BB/W-rat

Summary The spontaneously diabetic BB/W-rat has emerged as an important model system for somatic and autonomic diabetic polyneuropathy. In this study we examined visual evoked potentials and the presence of morphometric and structural changes in the optic nerve and the retinal ganglion cells and their afferent axons contained in the retinal nerve fibre layer. A six-month duration of diabetes mellitus was associated with significant increases in the latencies of the visual evoked potentials. The latency of the first positive potential showed a 44% increase, and that of the first negative potential was prolonged by 41%. No significant changes were demonstrated at any of the amplitudes. In the optic nerve mean myelinated fibre size was significantly reduced to 82% of control values, which was accounted for by a significant reduction in axonal size. Axo-glial dysjunction, a prominent structural defect of diabetic somato-sensory neuropathy in both man and diabetic rodents, was non-significantly increased in the optic nerve. In diabetic animals retinal ganglion cells displayed dystrophic changes. No such changes were observed in age- and sex-matched control animals. Proximal axons of the retinal nerve fibre layer showed an increase in dystrophic axons in diabetic BB/W-rats. Morphometric analysis of optic nerve capillaries revealed no abnormalities except for basement membrane thickening. The present data suggest that the diabetic BB/W-rat develops a central sensory neuropathy, characterized functionally by prolonged latencies of the visual evoked potentials and structurally by an axonopathy of optic nerve fibres.This study was presented in part at the 3rd International Workshop on Lessons from Animal Diabetes, Tokyo, Japan, September, 1990     

Effects of hyperglycaemia on visual evoked potentials in insulin-dependent diabetic patients

Multimodality evoked potentials frequently reveal subclinical involvement of the central nervous system in patients with insulin-dependent diabetes mellitus. We devised this study to evaluate the possible effects of acute hyperglycaemia on visual evoked potential (VEP) parameters in type 1 diabetic patients. A hyperglycaemic clamp (250 mg/dl for 180 min) was performed in ten patients. Monocular pattern reversal VEPs (check size 15, contrast 50%) were recorded before, and every 30 min after the start of the clamp. Basal VEP latencies and amplitudes were normal bilaterally in nine patients. No significant changes in pattern reversal and flash VEP parameters were observed after the induction or during the clamp period. None of the neurophysiological parameters evaluated during the test was related to the duration of the disease, the basal VEP latency or amplitude or the presence of retinopathy. Our data suggest that the neurophysiological abnormalities detected in insulin-dependent diabetic patients are due to structural involvement of the central nervous pathways and not to functional damage induced by acute short-term hyperglycaemia.     

Visual evoked potentials in insulin-dependent diabetics

Summary The latency of pattern-reversal VEPs was studied in type I insulin-dependent diabetics without retinal and extraocular involvement. One hundred eyes of 50 diabetics (mean age 19.8±7.2 years) formed the study group; the duration of the disease ranged between 1 month and 15 years. The latencies of VEPs were progressively delayed in relation to the duration of the disease, becoming more and more evident and stabilizing after about 6 years from the onset of diabetes. The VEP alterations probably indicate alteration of membrane balance or demyelinization.     

Brainstem auditory and visual evoked potentials in Type 1 (insulin-dependent) diabetic patients

Summary Brainstem auditory evoked potentials and pattern shift visual evoked potentials were measured in 34 Type 1 (insulin-dependent) diabetic patients with long-standing disease and in 43 control subjects. Thirty-two percent of diabetic patients had abnormal brainstem auditory evoked potentials and 15% had abnormal visual evoked potentials. These abnormalities were not related to duration of diabetes, diabetic control or individual diabetic complications (retinopathy, nephropathy, peripheral or autonomic neuropathy). The aetiology of the abnormalities must remain a subject for speculation. The findings of this study are consistent with a central diabetic neuropathy involving the brainstem in long-standing diabetic patients.     

Protective effects of cerebrolysin in a rat model of optic nerve crush

To investigate the effects of cerebrolysin (Cbl) on optic nerves (ON) and retinal ganglion cells (RGC) in a rat model of ON crush. Rats received intravitreal injection of Cbl (n = 20), intra-ON injection of Cbl (n = 20), intraperitoneal injection (IPI) of Cbl (n = 20), or phosphate buffered saline (PBS; n = 20) every day for 2 weeks after ON crush injury. At 3 weeks post-trauma, RGC density was counted by retrograde labeling with FluoroGold and visual function was assessed by flash visual-evoked potentials. Activities of microglia after insults were quantified by immunohistochemical analysis of the presence of ED1 in the optic nerve. At 3 weeks postcrush, the densities of RGCs in the Cbl-IVI group (1125 ± 166/mm²) and in the Cbl-IPI treatment group (1328 ± 119/mm²) were significantly higher than those in the PBS group (641 ± 214/mm²). The flash visual-evoked potential measurements showed that latency of the P1 wave was significantly shorter in the Cbl-IVI- and Cbl-IPI-treated groups (105 ± 4 ms and 118 ± 26 ms, respectively) than in the PBS-treated group (170 ± 20 ms). However, only Cbl IPI treatment resulted in a significant decrease in the number of ED1-positive cells at the lesion sites of the ON (5 ± 2 cells/vs. 30 ± 4 cells/high-power field in control eyes). Treatment with intra-ON injection of Cbl was harmful to the optic nerve in the crush model. Systemic administration of Cbl had neuroprotective effects on RGC survival and visual function in the optic nerve crush model.     

Cortical and spinal evoked potential response to electrical stimulation in human rectum

AIM: To study a novel technique to record spinal and cortical evoked potentials (EPs) simultaneously in response to electrical stimulation in the human rectum. METHODS: Eight male and nine female healthy volunteers participated. Stimulating electrodes were attached to the rectal mucosa at 15 cm and 12 cm above the dentate line. Recording skin electrodes were positioned over vertebrae L4 through S2. The electrical stimulus was a square wave of 0.2 ms duration and the intensity of the stimulus varied between 0 and 100 mA. EP responses were recorded using a Nicolet Viking IV programmable signal conditioner.RESULTS: Simultaneous recording of cortical and spinal EPs was obtained in > 80% of the trials. The EP responses increased with the intensity of the electrical stimulation, were reproducible overtime, and were blocked by application of Lidocaine jelly at the site of stimulation. The morphology (N1/P1), mean ± SD for latency (spinal N1, 4.6 ± 0.4 ms; P1, 6.8 ± 0.5 ms; cortical N1, 136.1 ± 4.2 ms; P1, 233.6 ± 12.8 ms) and amplitude (N1/P1, spinal, 38 ± 7 μV; cortical 19 ± 3 μV) data for the EP responses were consistent with those in the published literature. Reliable and reproducible EP recordings were obtained with the attachment of the electrodes to the rectal mucosa at predetermined locations between 16 and 8 cm above the anal verge, and the distance between the attachment sites of the electrodes (the optimal distance being approximately 3.0 cm between the two electrodes). CONCLUSION: This technique can be used to assess potential abnormalities in primary afferent neural pathways innervating the rectum in several neurodegenerative and functional pain disorders.     

Visual evoked potentials in NIDDM: a longitudinal study

Summary In order to assess the possible progression of neurological abnormalities over time and the value of visual evoked potential alterations in predicting stability and severity of diabetes-related optic pathway disease, a longitudinal study in non-insulin-dependent diabetic patients was performed. Neurological examination, visual evoked potentials with pattern reversal, motor and sensory nerve conduction velocities and metabolic control were studied in 18 non-insulin-dependent diabetic patients and in 35 normal control subjects at baseline and again after 4.6±0.8 years (range 4–6). At the first recording the peak P100 wave latencies were significantly delayed in the diabetic patients compared with the control subjects; signs of peripheral neuropathy were detected in five patients, clinical in three and in two there was only neurophysiological alteration without clinical signs. The second recording revealed no significant alterations of P100 latencies in patients compared with baseline, but the number with clinical signs and/or neurophysiological alterations with no clinical signs of peripheral neurological disease was increased to seven. In conclusion, we observed that visual evoked potential alterations were stable over time whereas peripheral neurological disease progressed and correlated positively with metabolic control.Abbreviations VEP-PR Visual evoked potentials with pattern reversal - NCV nerve conduction velocities - LP100 peak latencies of P100 wave - ILD interocular latencies difference - NIDDM non-insulin-dependent diabetes mellitus - ARI aldose reductase inhibitor     

Cerebral evoked potentials

Cerebral evoked potentials (EPs) represent a new technique for the evaluation of afferent outflow from the gastrointestinal tract. We compared EPs obtained with distension of the distal and proximal esophagus. Responses were recorded with the balloon 5 cm proximal to the lower esophageal sphincter and 3 cm distal to the upper esophageal sphincter. Balloon stimulation resulted in cortical responses recorded by midline scalp electrodes (CZ, PZ, and OZ by the International 10–20 system) in normal volunteers. EP responses consisted of two negative (N1, N2) and one positive (P1) deflections. The proximal esophageal latency of N1 was shorter in all three leads. The latency to P1 was shorter with proximal stimulation in lead CZ only, and N2 latencies were not different. Amplitudes expressed as the difference between N1 and P1, and P1 and N2 were not different. When two sets of potentials several minutes apart from the proximal position were compared, a decrease in amplitude with the second set of stimulations was noted. Esophageal EP recording is a new technique that may provide information about the integrity and function of the sensory innervation of the esophagus.Supported in part by a research grant to Dr. DeVault from the American College of Gastroenterology.