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.     

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。     

Hydrodynamic aspects of pyelocalyceal peristalsis

This study was undertaken to determine the nature of pressure changes in manometric studies of renal pelvis and calyces. In previous studies in pigs it has been assumed that pressure increases occur in the contracting segments of the renal pelvis and calyces, but our observations suggested that these increases were actually due to distension of noncontracted segments. Pressures were recorded with two catheters introduced percutaneously into the pyelocalyceal system with simultaneous video recording of the fluoroscopic image. There was no pressure rise in the calyces or the renal pelvis when these segments contracted; however, pressure rose when the noncontracted calyces were distended by a remote contraction ring. These findings confirmed the observations at kinetic urography. They agreed with the hydrodynamic experience that the pressure in a ring-shaped contracted segment is lower than upstream and downstream of that segment. It was concluded that if the porcine pyelocalyceal system is a valid model of the human, the pressure increases, assumed to be contractions, were actually caused by the injection and distension of the noncontracted segments.     

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     

电针内关穴对窦房传导功能的影响及其意义

采用直接窦房传导时间（ＳＡＣＴｄ）为指标，对１０例窦房结功能正常者进行了针刺前、针刺及固有心率（ＩＨＲ０）期间ＳＡＣＴｄ测定，以探讨电针内关穴对窦房传导功能的影响及其意义。结果表明：电针与ＩＨＲ０期间ＳＡＣＴｄ较对照期显著缩短（Ｐ〈０．０１；Ｐ〈０．００１）。自主神经阻滞后，电针该穴不能使ＳＡＣＴｄ进一步缩短（Ｐ〉０．０５）。提示；以内关穴为主的电针刺激能改善正常窦房传导功能，其作用似与自主神经中     

126例心房颤动合并长R-R间期患者的动态心电图分析

Objective To analyze the relationship between atrial fibrillation complicating long R-R interval, escape beat and escape heart rhythm and pathological atrioventricular block by dynamic electrocardiography (DCG). Methods 126 patients with atrial fibrillation were divided into group A (being related to sleep) and group B (being not related to sleep) according to the factor whether long R-R interval, escape beat and escape heart rhythm relevant to sleep. All were detected by DCG for 24 hours. Results The cases with1.5～2.0 s long R-R interval, more than 2.0s long R-R interval, escape beat and escape heart rhythm were (26.02±6.03), (7.39±1.05 ) and (6.90±1.28)in group A and (203.05±41.01), (35.48±7.52), (28.10±6.25) in group B respectively. The cases of group B were markedly more than those in group A. Conclusion Those atrial fibrillation complicating long R-R interval, escape bbeat and escape heart rhythm were related to sleep could not be deemed as pathological atrioventricular block, while those were not related to sleep could be considered pathological atrioventricular block.     

Effects of complete heart block on myocardial function, morphology, and energy metabolism in the rat.

AIMS: Severe sustained bradycardia may cause acute and possibly chronic congestive heart failure (CHF). The aim of this study was to investigate acute and chronic effects of complete heart block (CHB) on cardiac function, morphology, and creatine (Cr) metabolism. METHODS AND RESULTS: CHB was induced in male Sprague-Dawley rats (approximately 250 g, n = 11) by means of electrocautery applied to the region of AV node and were compared with controls (n = 15). The rats were investigated at 1, 3, and 12 weeks after CHB induction with transthoracic echocardiography. Invasive haemodynamic assessment of left and right ventricular pressures was performed at 12 weeks. After the sacrifice, the hearts were freeze-clamped for analysis of myocardial Cr, and high energy phosphometabolites. The efficacy of operative procedure was 54%. The peri-operative mortality rate was 20%. Heart rate (HR) decreased by approximately 50% (P < 0.01) while stroke volume (SV) increased 2.5 times (P < 0.01) in the CHB rats. Cardiac index remained unchanged. The rats with CHB grew normally and were in no apparent distress. Filling pressures in left and right ventricles were normal. The CHB rats developed marked cardiomegaly with biventricular dilatation and eccentric left ventricular hypertrophy (P < 0.01). There was no change in the myocardial content of Cr and high energy phosphometabolites. CONCLUSION: Rats with CHB are compensating for reduction in HR with increased SV without haemodynamic and biochemical characteristics of CHF. This model may be useful to study the effects of CHB and bradycardia on myocardial structure, function, electrophysiology, and metabolism as well as for studies of cell therapy for reparation of AV conductance.