Ritually induced growth disturbances and deformities of the orofacial system – A contribution to cranial morphogenesis

Numerous ritual acts involving the skull result in orofacial changes. The present study focuses on ritual acts of Brazilian Zoé Indians. A distinct deformation effect of the ritual act (wearing a lip-plug) on the morphology of the orofacial system is demonstrated and documented using jaw models. The studies show that the lip-plug significantly influences tooth position and jaw growth. While the maxilla displays palatal displacement of the lateral incisors and elevation of the palate, retraction occurs in the mandible depending upon plug size. Additionally, both the plug and the nutritional habits of the Indians induce marked abrasion of all teeth. Moreover, it is shown that the duration of lip-plug wear is an essential determinant of sustained orofacial changes.     

抗磷脂抗体对妊娠的影响

自身免疫性疾病或免疫功能异常影响妊娠，在自身免疫疾病中，抗磷脂综合征（antiphospholipid syndrome APS）占了一定比例。本文就抗磷脂抗体的性质进行详细阐述。血栓形成是APS的主要病理基础和突出临床表现，随着研究深入，APA对早孕期滋养细胞的影响越来越受到重视，而APA导致不良妊娠结局的作用机制，目前仍有争论。本文还介绍了APS最新的治疗进展，为今后的APS发病机制研究及临床治疗带来更多的启示。     

Comparison of salivary calmodulin binding proteins in Sjögren''s syndrome and healthy individuals

Background: Reduction in salivary secretion is the hallmark of Sjögren's syndrome (SS). Calmodulin (CaM) and calmodulin binding proteins (CaMBPs) play a key role in the secretory process of saliva. Recent studies have suggested that SS‐B, an autoantibody associated with SS, is a CaMBP. This finding suggests that CaMBP may contribute to the loss of saliva in SS. To better understand the role(s) of these proteins in SS, the purpose of this study was to compare salivary CaMBPs in Sjögren's patients and controls. Methods: Saliva samples were collected from 20 patients and 20 age‐, race‐, and gender‐matched controls. CaM overlay was used to identify CaMBPs in saliva of patients and controls. Results: Higher number of salivary CaMBPs was observed among patients than controls. Conclusions: The increased number of salivary CaMBPs in SS may suggest a potential role for these proteins in the pathogenesis of the disease.     

Urgent coronary artery bypass surgery in the presence of Dressler's syndrome

The clinical features of Dressler's syndrome which developed in a patient following a third myocardial infarction responded to a short course of prednisolone. However, angina at rest precipitated urgent coronary artery bypass surgery and pericardectomy.     

Granulocytes in bronchial lavage fluid after major vascular surgery

Increased numbers of polymorphonuclear granulocytes (PMN) in the airways, as measured by PMN content in bronchial lavage fluid (P less than 0.01), were found 3 h postoperatively in ten patients undergoing surgery for lumbar aortic aneurysms. An increase in plasma levels of the complement split product C3dg from 6 (0-19) AU/ml preoperatively to 20 (13-50) AU/ml 3 h after surgery (P less than 0.01), indicates an activation of the complement cascade. These changes were not accompanied by increased elastase activity in the bronchial lavage fluid or by major changes in pulmonary blood gas exchange or vascular resistance, indicating that massive PMN activation, analogous to that proposed in adult respiratory distress syndrome (ARDS) had not taken place. In conclusion, complement system activation and migration of PMN into the airways, as seen in connection with major vascular surgery, does not seem to contribute to ARDS-type pulmonary dysfunction.     

肝素治疗小儿肾病综合征的远期效应

采用无抗凝作用剂量肝素皮下注射治疗小儿肾病综合征２５例，观察了近期疗效与１２～２４月后对皮质激素或环磷酰胺（ＣＴＸ）治疗反应的关系．结果表明，近期对肝素治疗显效但复发以及有效者，今后用激素或ＣＴＸ治疗均可达完全缓解，无效者则效果较差．本文结果表明，肝素治疗的近期疗效可用于预测肾病患儿的远期预后．     

Uveal effusion syndrome complicated by anterior ischemic optic neuropathy

We report on a case of idiopathic uveal effusion syndrome complicated by AION. To our knowledge such an association hasn't been previously described. We suggest that scleral thickening caused obstruction of vortex veins followed by uveal effusion and compression of posterior ciliary arteries within their intrascleral tract, leading to AION. Nevertheless it can't be excluded that AION was the result of mechanical compression on ciliary vessels of optic disc by choroidal detachment.     

Heart rate variability and apnea during sleep in Down's syndrome

Autonomic system dysfunction has been reported to occur frequently in patients with Down's syndrome (DS) and is constituted mainly by an imbalance between the sympathetic and vagal systems. The analysis of heart rate variability (HRV) during sleep is a quantitative reliable method for studying such a mechanism, but it has not yet been extensively and adequately applied in DS. In this study, HRV during sleep was evaluated in seven DS patients and in six normal controls, by also controlling for the presence of sleep apnea or arousal. The main results were an increased sympathetic function (low-frequency component of HRV) and a decreased vagal activity (high-frequency component of HRV) in DS with respect to normal controls, during apnea-free periods. Moreover, the presence of apnea, in DS, induced a further significant increase in low-frequency and very low-frequency components of HRV during sleep Stage 2. This study provides additional evidence of a brainstem dysfunctioning in DS, responsible for the abnormal imbalance between the sympathetic and vagal systems and confirms the brainstem involvement already suggested in the literature in order to explain brainstem-auditory evoked potential abnormalities and central sleep apnea in these patients.     

Reduced genital sensitivity in female patients with multiple system atrophy of parkinsonian type.

According to the consensus statement on the diagnosis of multiple system atrophy (MSA), erectile dysfunction is required for male patients to fulfil the urinary incontinence criterion. However, there is no equivalent item for female patients. We questioned 19 female patients with MSA of the parkinsonian type (MSA-P), 28 female patients with Parkinson's disease (PD), and 27 healthy controls on their genital sensitivity. A total of 47% of the MSA patients but only 4% of the PD patients and 4% of the control group admitted to reduced genital sensitivity, a highly significant difference (P < 0.001). Moreover, the appearance of reduced genital sensitivity in female MSA patients showed a close temporal relation to the onset of the disease. If these preliminary results can be confirmed and further specified in a larger sample, a historical item of reduced genital sensitivity in female patients might become a diagnostic feature for MSA, comparable to erectile dysfunction in male patients.     

Absence of predictable phenotypic expression in proximal 15q duplications

We describe ten individuals with an insertional duplication 15q12----q13. Phenotypic analysis of these individuals and 15 previously reported cases of proximal 15q duplications fails to show any consistent clinical manifestations. It appears that a duplication of this region is phenotypically silent.