A Ro/SS-A auto-antibody positive mother's infant revealed congenital complete atrioventricular block,followed by insulin dependent diabetes mellitus and multiple organ failure

We experienced a congenital complete atrioventricular block infant who was born from a Ro/SS-A antibody positive mother. Ro/SS-A antibody was also found in this baby which was presumed to be mediated by the maternal placenta. Temporary cardiac pacing was required at birth and pacemaker implantation was performed at 9 months. At 11 months of age, the baby fell into shock and experienced multiple organ failure because of diabetes mellitus-induced coma. The association between congenital complete heart block and the Ro/SS-A antibody is well known. However, the accompaniment of insulin-dependent diabetes mellitus has not been reported previously. As the Ro/SS-A antigen appears in the cytoplasm of many tissues, the possibility of an association between Ro/SS-A antibody and diabetes mellitus is difficult to deny. We report this rare case to draw attention to the possibility that babies who are born from an Ro/SS-A antibody positive mother may develop diabetes mellitus as well as congenital complete heart block.     

Ultrasound biomicroscopic features of spherophakia

Background: Spherophakia is an uncommon diagnosis. This is the first case report of spherophakia evaluated by ultrasound biomicroscopy.
Methods: Ultrasound biomicroscopy is a new diagnostic technique developed by one of the authors and provides images with microscopic resolution of the anterior segment. A patient with spherophakia was evaluated by ultrasound biomicroscopy (Zeiss-Humphrey, 50MHz) before and after YAG laser iridotomy.
Results: Ultrasound biomicroscopic assessment revealed a shallow anterior chamber, a very steep anterior lens curvature, iridolenticular contact, elongated zonules, and an increased distance between the lens equator and the ciliary processes. Angle closure glaucoma was due to a pupil block mechanism. The pupil block was relieved by YAG laser iridotomy.
Conclusions: Ultrasound biomicroscopy is a useful technique to confirm the diagnosis of spherophakia. The pupil block in spherophakia is relieved by YAG laser iridotomy.     

动态MRI对臂丛根性损伤和颈髓损伤的鉴别诊断意义

目的探讨动态MRI诊断臂丛神经根性损伤的影像学表现及其诊断价值，以及在与颈髓损伤的鉴别诊断中的临床意义。方法对8例臂丛神经根性损伤患者行动态MRI检查，结合CT薄层扫描和肌电图随访；所有8例患者均手术证实为臂丛根性损伤。结果本组患者在伤后早期1～2d内均无位于椎间孔及椎间孔外的创伤性脊膜囊肿、神经根断裂等支持臂丛根性损伤的MRI异常表现；4例患者有颈椎骨折或颈髓损伤的MRI表现。伤后7～15d（平均10d）后，再次行颈椎MRI检查，出现典型的MRI臂丛根性损伤表现：椎间孔及椎孔外臂丛神经走行处T2WI高信号的创伤性脊膜囊肿、脊髓移位和神经根缺失及走行异常等。结论动态MRI检查对臂丛根性神经损伤具有损伤节段定位准确、椎管外部分同样能够显示、无创、操作简单、准确性高等特点；其短期内特征性的动态变化易于诊断臂丛根性损伤，同时又能与颈髓损伤相鉴别。     

双针会师法腹腔丛阻滞对顽固性上腹部癌痛患者的镇痛作用

晚期癌痛患者17例，男9例，女8例，年龄46—85岁，体重38—63kg，VASⅢ或Ⅳ级。CT引导下经T12-L1椎间隙左、右穿刺，针尖分别抵达膈脚外腹主动脉旁及膈脚内。分别注入含造影剂（30％碘海醇注射液1ml）的1％利多卡因8ml，CT示所注药液有会师趋势或已会师包绕腹主动脉后，左、右分别注入无水酒精20、15ml，再次CT观察酒精扩散情况，以视觉模拟评分（VAS）及改良生活质量（QOL）评分评价镇痛效果。会师包绕腹主动脉的11例患者中8例（73％）镇痛效果满意至去世；另6例酒精在腹主动脉周围被转移的淋巴结分割呈斑点、斑片状，其中3例（50％）完全无痛。与腹腔丛阻滞（NCPB）前相比，NCPB后各时点的VAS评分下降，QOL评分升高（P〈0．01）。双针会师法腹腔丛阻滞可使所注无水酒精在腹腔丛恰当分布，对顽固性上腹部癌痛患者有较好的镇痛效果。     

CLINICAL IMPLICATIONS OF ELECTROPHYSIOLOGY STUDY FINDINGS IN PATIENTS WITH CHRONIC BIFASCICULAR BLOCK AND SYNCOPE

Electrophysiology study was performed in 93 patients with bifascicular block and unexplained syncope. Clinical evidence of organic heart disease was present in 33 (35%). Electrophysiological abnormalities were detected in 45 patients (48%). Of these, 36 had distal conduction disease, including 28 with an HV interval > 55 ms (mean 76.4 ms), and eight who developed infraHisian block following either intravenous procainamide (four) or atrial pacing (four). Sick sinus syndrome was evident in six patients and a further two had carotid sinus hypersensitivity. Sustained monomorphic ventricular tachycardia (VT) was induced in only three patients, two of whom also had prolonged HV interval. Among the 93 patients, 45 had therapy which was guided by positive findings at electrophysiology study (Group 1). Of these, 42 received permanent pacemakers, two were treated with combined permanent pacing and antiarrhythmic drug therapy, and one was treated with antiarrhythmic drug alone. In addition, eight patients without electrophysiologic abnormalities were treated empirically by pacing (Group 2). Finally, 40 patients without electrophysiologic abnormalities received no specific therapy (group 3). At a mean follow-up of 39 months (range two-125 months), recurrence of syncope had occurred in 4% of Group 1 patients, and 25% of Group 3 patients (p < 0.05). No patient in Group 2 had had recurrence. Total mortality was 40%, including 47% of patients in Group 1, 25% of Group 2, and 35% of Group 3. Death was sudden in seven patients. We concluded that among patients with bifascicular block and syncope, therapy directed by findings at electrophysiology study was associated with symptomatic improvement, but mortality was not significantly influenced. Patients with no electrophysiological abnormality have a high recurrence of syncope which may relate either to undetected bradyarrhythmia or ventricular tachycardia. These results highlight some limitations in current assessment, including, for programmed ventricular stimulation, significant differences in both stimulation protocols and the definition of ventricular tachycardia.     

Self-inflicted tourniquet paralysis mimicking acute demyelinating polyneuropathy.

Tourniquet paralysis is an uncommon complication of surgery, and self-inflicted tourniquet paralysis has never been documented to our knowledge. We report a patient with bilateral self-induced tourniquet paralysis of the lower extremities, whose symptoms were initially attributed to an acute demyelinating sensorimotor polyneuropathy based on clinical presentation and electrodiagnostic study. After investigations failed to reveal a cause, he was found to have placed tourniquets on his legs because of a rare obsession with limb amputation known as apotemnophilia. Significant spontaneous partial resolution of clinical symptoms was noted after 6 weeks. Electrophysiologic evidence of segmental demyelination of multiple motor nerves localized to the same region may help to distinguish this condition from other forms of acute demyelinating polyneuropathy.     

Rest thallium-201 myocardial perfusion imaging in a patient with leukaemic infiltration of the heart

Despite the high incidence of leukaemic infiltration of the heart, only 8 cases of atrioventricular block due to leukaemia have been reported in the literature. Improvement in the heart block associated with disappearance of the leukaemic infiltrate has not been reported. A rest thallium-201 study was used in a 65-year-old man to demonstrate leukaemic infiltration of the heart which was associated with complete heart block. After chemotherapy, when the tumour burden was reduced and the leukaemia in remission, his heart block resolved, and a follow-up thallium scan was normal. Offprint requests to: A.C. Civelek     

Effect of the stable thromboxane derivative, carbocyclic thromboxane A2, on membrane potential of rat myenteric neurones in culture

The effects of carbocyclic thromboxane A(2) (cTXA(2); 10(-6) mol L(-1)) on membrane potential and cytosolic Ca(2+) concentration were measured with the whole-cell patch-clamp or the fura-2 method, respectively, at rat myenteric ganglia. cTXA(2) caused a hyperpolarization of myenteric neurones from -19.3 +/- 2.5 to -29.3 +/- 2.3 mV. In addition, the eicosanoid potentiated the carbachol-induced depolarization from 4.2 +/- 1.0 mV under control conditions to 11.1 +/- 1.1 mV in the presence of the cTXA(2) (n = 9). The hyperpolarization was abolished by internal application of CsCl (140 mmol L(-1)), a non-selective blocker of K(+) channels, or EGTA (11 mmol L(-1)in the pipette solution), a chelator of intracellular Ca(2+). A similar inhibition was observed in the presence of charybdotoxin (10(-7) mol L(-1)). Fura-2 imaging experiments revealed a cTXA(2)-evoked increase in the intracellular Ca(2+) concentration as indicated by a rise in the fura-2 ratio signal. This response was mediated by a release of Ca(2+) from intracellular stores as sarcoplasmic-endoplasmic reticulum Ca(2+)-ATPase blockade with cyclopiazonic acid (5 x 10(-5) mol L(-1)) completely abolished the response to cTXA(2). A similar inhibition was observed after blockade of phospholipase C with U-73122 (10(-5) mol L(-1)). These results suggest an activation of Ca(2+)-activated K(+) channels by cTXA(2) after stimulation of phospholipase C.     

臂丛神经鞘瘤的MRI诊断

目的 探讨磁共振成像（magnetic resonance imaging，MRI）在臂丛神经鞘瘤诊断中的应用价值。方法 分析36例经手术和病理证实的臂丛神经鞘瘤术前MRI表现。观察内容包括病变部位、肿块大小、边界、信号、强化特征及范围。结果 MRI能清晰显示臂丛神经的根、干及分支，表现为线状低信号影。36例臂丛神经鞘瘤平均直径为4．9cm，均表现为沿臂丛神经根生长的肿块，其中30例表现为边界清楚的纺锤形或长圆形肿块。MRI显示．16例表现为均匀的T1WI低或等信号（与肌肉相比），T2WI高信号，增强后中等度强化；20例T1WI呈低信号，T2WI呈不均匀高信号，内见低信号区，所谓的“靶征”，增强后明显强化。结论 臂丛神经鞘瘤具有特征性的发生部位、形态学表现及MRI信号特点，对本病能提供准确的诊断依据。