POEMS综合征8例临床分析

目的探讨POEMS综合征发病机制及临床特征，提高对POEMS综合征的诊治水平。方法分析8例POEMS综舍征的一般资料、临床表现、相关的辅助检查、治疗及预后，并结舍文献进行讨论。结果本病是累及多系统的疾病，以周围神经病最具有特征性，肝、脾和（或）淋巴结肿大，内分泌的病变，皮肤改变，水肿及骨髓浆细胞增多比较常见。治疗以激素、免疫抑制剂为主，可结合抗雌激素、放疗的方法，预后差。结论POEMS综合征与浆细胞病关系密切，累及多系统的病变，临床表现复杂多样，应注意多系统的检查，提高临床的诊断水平。     

白细胞对早期糖尿病视网膜病变作用的研究

目的探讨白细胞在早期糖尿病大鼠视网膜毛细血管中粘附、堆积及其与视网膜微血管形态学变化的关系。方法健康成年雄性Wistar大鼠90只，随机分成正常对照组和链脲佐菌素（streptozotocin, STZ）诱导的糖尿病组各45只（3、7、14 d组各5只，30、90、180 d组各10只）。取大鼠右眼制备视网膜消化铺片，进行形态学观察及白细胞共有抗原（leukocyte common antigen, CD45）单克隆抗体免疫组织化学研究。结果正常对照组视网膜毛细血管中有少许CD45阳性细胞；糖尿病发生3 d后，CD45的表达明显增加，病程第14 d达高峰，以后基本稳定。病程90 d时，视网膜毛细血管已出现节段性膨大、囊样扩张及闭锁等形态学改变；病程180 d时，上述病变进一步加重。结论白细胞粘附发生在糖尿病视网膜病变（diabetic retinopathy, DR）的早期阶段，是DR在微血管水平病理变化的开端。白细胞粘附可能作为视网膜微血管形态学改变的基础，在DR的发生发展中起着重要作用。(中华眼底病杂志,2003,19:344-347)     

Novel C59T leader peptide mutation in the MPZ gene associated with late-onset, axonal, sensorimotor polyneuropathy

The objective of this study was to report a novel exon-1 mutation in the myelin protein zero (MPZ) gene, resulting in axonal Charcot–Marie–Tooth neuropathy with recurrent hyper-CK-emia. In a 64-year-old woman slowly progressive distal lower limb weakness, muscle cramps in the lower limb muscles, and stocking-type numbness had developed from the age of 61. Neurologic examination revealed discrete hip flexor weakness, weakness for foot extension, diffuse wasting of the distal lower limb muscles, reduced patella tendon reflexes, and absent Achilles tendon reflexes. There was recurrently elevated creatine kinase with a maximum of 607 U/l ( n , <145 U/l). Stimulation of the peroneal and tibial nerves did not evoke a muscular response. Electromyography was neurogenic. Biopsy of the right sural nerve showed diffuse axonal degeneration and loss of axons of all diameters. Muscle biopsy showed increased fiber-size variability, angulated fibers, internalized nuclei, accumulations of nuclei, grouped atrophic muscle fibers, and fiber splitting. Molecular genetic analysis by PCR and direct nucleotide sequencing revealed the heterozygous C59T exon-1 MPZ gene mutation, resulting in the amino acid exchange S20F of the MPZ signal protein domain (leader peptide). The novel C59T mutation in the leader peptide of the MPZ gene is pathogenic and manifests as severe, late-onset, axonal, symmetric sensorimotor polyneuropathy (CMT2) and hyper-CK-emia.     

Anterior eye focusing of peripheral ultraviolet and visible radiation albedo

Concern about short- and long-term ultraviolet radiation (particularly UVB) damage to the eye has led to increased research in this area. Numerous studies have confirmed the pathogenic enhancing roles of reflected ultraviolet (UV) and visible radiation in our environment. There is concern that conventional sunglasses do not protect the eye adequately from reflected rays (albedo), especially on the lateral aspect, from behind and from below. Using eye models and computer ray tracing methods, the pathways of oblique rays incident at the temporal peripheral cornea have been plotted by Maloof, Ho and Coroneo.¹ These rays are refracted and focused and theoretically can result in up to 20 times the concentration of incident irradiance at the nasal anterior chamber angle and nasal equatorial cortex of the crystalline lens. The purpose of this study was to determine the limits of angular subtense of the incident peripheral light which is refracted in this manner in human subjects and to investigate the relation between corneal shape and certain ocular parameters to the limits. A statistically significant positive correlation was found between temporal entrance angle and anterior chamber depth (r = 0.70, P< 0.0006). The entrance angle ranged from 15 degrees to 30 degrees and was located 10 degrees to 45 degrees posterior to the coronal plane. Our results support Maloof and colleagues' predictions for the implication of focused peripheral UV and high intensity visible radiation in the pathogenesis of pterygium and cortical cataract and emphasise the need for lateral eye protection in conditions of high ultraviolet albedo.     

疏血通对早期糖尿病肾病的治疗作用

目的观察疏血通对糖尿病肾病(DN)的治疗作用。方法选择早期DN病人62例随机分为疏血通治疗纽和对照组,对照组行饮食控制及应用胰岛素或糖适平控制血糖。治疗组在上述治疗基础上加疏血通治疗共4周。观察两组治疗前后血糖、血常规、血生化、肝功能、肾小球滤过率(GFR)、尿清蛋白排泄率(UAER)及治疗过程中出现的副作用。结果治疗前两组病人GFR,UAER相近,治疗后两组病人GFR,UAFAt明显改善(3.051-5.126,P<0.05),而治疗组上述指标改善优于对照组(t=2.105,2.864,P<0.05),疏血通治疗过程中无明显的副作用发生。结论疏血通对早期糖尿病肾病有明显疗效,值得推广应用。     

Sudden cardiorespiratory arrest after renal transplantation in a patient with diabetic autonomic neuropathy and prolonged QT interval

A 31–yr male with insulin dependent diabetes mellitus for 20 years underwent general anaesthesia for renal transplantation. During transfer from operating theatre to ICU he developed bradycardia advancing to ventricular fibrillation and had to be resuscitated. Bradycardia did not respond to atropine. Postoperative autonomic nervous function tests showed advanced autonomic neuropathy. He was found to have constantly prolonged QTc interval in his pre– and postoperative ECGs (462–503 ms). Prolongation of QTc interval could be used as a valuable predictor of postoperative cardiac complications in diabetic patients with autonomic neuropathy.     

Neuropathy associated with “benign” anti-myelin-associated glycoprotein IgM gammopathy: Clinical,immunological, neurophysiological pathological findings and response to treatment in 33 cases

We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46–81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG.     

前列腺素与糖尿病肾病关系的探讨

用放免法测定糖尿病肾病２６例、单纯糖尿病２４例和同年龄的健康对照者２５例血浆的血栓素Ｂ２（ＴＸＢ２）、６－酮前列腺素Ｆ１α（６－ｋ－ＰＧＦ１α）及空腹血糖（ＦＳＧ）、基础胰岛素（ＩＦ）、血肌酐（ＳＣｒ）水平。结果显示，糖尿病肾病组ＴＸＢ２明显高于对照组，６－ｋ－ＰＧＦ１α明显下降，ＳＣｒ与－ｋ－ＰＧＦ１α呈负相关，与ＴＸＢ２呈正相关，ＴＸＢ２／６－ｋ－ＰＧＦ１α与空腹血糖呈正相关，与内源胰岛素释放无关。     

Approved indications of lipo-PGE1 in Japan

For its peripheral vascular dilating effect and platelet agglutination inhibitory activity, prostaglandin E₁ is used in the treatment of diseases which are likely to cause peripheral circulatory failure or thrombus. In Japan, lipo-PGE₁, which was developed to give it a target-directed nature by modifying the conventional PGE₁, has been used and found to be useful in clinical practice. In this report, we attempt to describe the clinical benefits of lipo-PGE₁ focusing on the diseases which have been approved for its indications.