Atypical nasal challenges in patients with idiopathic rhinitis: more evidence for the existence of allergy in the absence of atopy?

BACKGROUND: The pathophysiology of idiopathic rhinitis is unknown although evidence is accumulating to suggest that many patients may have a localized form of allergic rhinitis in the absence of other atopic symptoms and markers. This study compares detailed nasal challenge results obtained from patients with idiopathic rhinitis to those of atopic and normal controls. METHODS: Patients with idiopathic rhinitis (n = 23), perennial allergic rhinitis (n = 8) and normal controls (n = 8) underwent a normal saline challenge to exclude hyper-reactivity and then bilateral nasal allergen challenges. Nasal patency was assessed by anterior active rhinomanometry. RESULTS: All of the patients with atopic rhinitis demonstrated positive bilateral allergen challenges. All normal control subjects had bilateral negative challenges. Two patients in the idiopathic group tested positively to saline and were excluded from further study with 62% of the remainder testing positive to allergens. Of the idiopathic patients testing positive, 85% were sensitive to house dust mite. CONCLUSION: A significant proportion of patients with idiopathic rhinitis have positive nasal challenges, the vast majority to house dust mite allergen. These findings add to the weight of evidence that suggests 'localized allergy' may exist in the absence of systemic atopic markers.     

Pathophysiology of idiopathic dystonia: findings from genetic animal models

Dystonia is a common movement disorder which is thought to represent a disease of the basal ganglia. However, the pathogenesis of the idiopathic dystonias, i.e. the neuroanatomic and neurochemical basis, is still a mystery. Research in dystonia is complicated by the existence of various phenotypic and genotypic subtypes of idiopathic dystonia, probably related to heterogeneous dysfunctions.In neurological diseases in which no obvious neuronal degeneration can be found, such as in idiopathic dystonia, the identification of a primary defect is difficult, because of the large number of chemically distinct, but functionally interrelated, neurotransmitter systems in the brain.The variable response to pharmacological agents in patients with idiopathic dystonia supports the notion that the underlying biochemical dysfunctions vary in the subtypes of idiopathic dystonia. Hence, in basic research it is important to clearly define the involved type of dystonia.Animal models of dystonias were described as limited. However, over the last years, there has been considerable progress in the evaluation of animal models for different types of dystonia.Apart from animal models of symptomatic dystonia, genetic animal models with inherited dystonia which occurs in the absence of pathomorphological alterations in brain and spinal cord are described.This review will focus mainly on genetic animal models of different idiopathic dystonias and pathophysiological findings. In particular, in the case of the mutant dystonic (dt) rat, a model of generalized dystonia, and in the case of the genetically dystonic hamster (dt^sz), a model of paroxysmal dystonic choreoathetosis has been used, as these show great promise in contributing to the identification of underlying mechanisms in idiopathic dystonias, although even a proper animal model will probably never be equivalent to a human disease.Several pathophysiological findings from animal models are in line with clinical observations in dystonic patients, indicating abnormalities not only in the basal ganglia and thalamic nuclei, but also in the cerebellum and brainstem. Through clinical studies and neurochemical data several similarities were found in the genetic animal models, although the current data indicates different defects in dystonic animals which is consistent with the notion that dystonia is a heterogenous disorder.Different supraspinal dysfunctions appear to lead to manifestation of dystonic movements and postures. In addition to increasing our understanding of the pathophysiology of idiopathic dystonia, animal models may help to improve therapeutic strategies for this movement disorder.     

Greenberg dysplasia (hydrops–ectopic calcification–moth‐eaten skeletal dysplasia): Prenatal ultrasound diagnosis and review of literature

A second pregnancy of young, nonconsanguineous parents of Macedonian ethnic origin was examined by ultrasound. Polyhydramnios and hydrops fetalis were found as well as severe short limb, short stature, and cystic hygroma of the neck. An artificial abortion was performed at the age of 23 weeks. The radiological features included moth‐eaten severely shortened long bones and ectopic calcifications of long bones, vertebral column, ribs, pelvis, larynx, trachea. In addition, the fetus had large head with depressed nasal bridge, severe platyspondyly, and short barrel‐shaped trunk. Light microscopy demonstrated lack of chondrocyte columns and disorganization of the cartilaginous architecture. This is the seventh reported case of this rare form of lethal skeletal dysplasia. © 2002 Wiley‐Liss, Inc.     

免疫性血小板减少性紫癜辨证分型与血小板表面相关抗体及T淋巴细胞亚群的关系

为探讨免疫性血小板减少性紫癜（ITP）中医辨证分型与免疫机理的关系,将ITP患者中医辨证分型结合血小板相关抗体（PAIg）及T淋巴细胞亚群进行分析。发现作为本病主要抗体的PAIgG脾肾阴亏型明显高于脾虚失统型（P<0.01）;OKT₈⁺值亦显著高于脾虚失统型（P<0.05）,OKT₄⁺/OKT₈⁺比值则明显低于脾虚失统型（P<0.05）。提示脾肾阴亏型的免疫损伤程度较脾虚失统型严重。     

Prevalence study of primary dystonia in Iceland.

In Iceland, the crude prevalence for all types of primary dystonia was 37.1/10(5) (confidence interval, 30.4-44.9). Focal dystonia had the highest prevalence (31.2/10(5)), followed by segmental (3.1/10(5)), multifocal (2.4/10(5)) and generalized dystonia (0.3/10(5)). Cervical dystonia was the most common focal dystonia (11.5/10(5)), followed by limb dystonia (8.0/10(5)), laryngeal dystonia (5.9/10(5)), blepharospasm (3.1/10(5)), and oromandibular dystonia (2.8/10(5)). The male:female ratio for all patients was 1:1.9 (P=0.0007), and females outnumbered males in all subtypes except oromandibular dystonia. Mean age of onset for all patients was 42.7 years (range, 3-82 years). This prevalence of primary dystonia is higher than in most reported studies, possibly because of more complete ascertainment but the relative frequencies of dystonia subtypes is similar.     

青少年特发性胸椎侧凸后路凸侧胸廓成形术的并发症分析

目的:探讨青少年特发性胸椎侧凸患者后路凸侧胸廓成形术的并发症发生原因及预防措施。方法:自1999年1月～2006年9月,共有410例青少年特发性胸椎侧凸患者在我科施行脊柱侧凸后路矫形内固定术,同时行同一切口下的凸侧胸廓成形术,男86例,女324例。年龄11～18岁,平均14.7岁。术前剃刀背畸形15°～48°,平均32°。统计并发症发生情况。结果:凸侧胸廓成形的肋骨切除数平均4.2根,术后剃刀背畸形0°～14°,平均6°。无手术死亡病例。24例(5.8%)术中发生壁层胸膜穿孔,6例(1.5%)术后胸腔积液,3例(0.7%)术后气胸,1例(0.2%)术后呼吸困难需间歇性吸氧,34例(8.3%)术后有局部反常呼吸,17例(4.1%)术后诉不同程度的胸壁疼痛,9例(2.2%)诉局部皮肤麻木或感觉减退,3例(0.7%)早期手术的患者术后肋骨残端突出明显,1例(0.2%)皮下血肿,1例(0.2%)皮肤压疮。经相应处理后,患者大多恢复满意。319例平均随访28个月,仅4例仍残留有胸壁皮肤感觉减退。结论:凸侧胸廓成形术是一种安全、有效的改善"剃刀背"畸形的手段。提高手术技巧,术后积极处理,可以减少甚至避免相关并发症的发生。     

Perceived adherence to prescribed treatment in juvenile idiopathic arthritis over a one‐year period

Objective

To document perceived adherence to treatment (taking medications and performing exercises) in patients with juvenile idiopathic arthritis (JIA) over a 1‐year period and to identify related factors.

Methods

We surveyed parents of patients with JIA at the Montreal Children's Hospital and British Columbia's Children's Hospital in Vancouver. Parents were asked to respond to a series of questionnaires every 3 months over a 12‐month period. Perceived adherence was evaluated on a 100‐mm visual analog scale (VAS) in the Parent Adherence Report Questionnaire (PARQ). Parental coping, distress, child function, disease severity and duration, perceived helpfulness of treatment, problems encountered, and sociodemographic data were also assessed.

Results

The mean age of our sample of 175 children was 10.2 years; mean age at diagnosis was 6.1 years and mean disease duration was 4.1 years. Perceived adherence to medications was consistently high, with average adherence at baseline, 3, 6, 9, and 12 months being 86.1, 91.7, 90.4, 92.0, and 88.8, respectively, on the PARQ VAS. Perceived adherence to exercise was lower but remained steady, with corresponding means of 54.5, 64.1, 61.2, 63.0, and 54.3, respectively. Using generalized estimating equation analysis, factors associated with higher perceived adherence to medications included perceived helpfulness of medications and lower disease severity; those associated with higher perceived adherence to exercise were younger age of the child, child involvement in responsibility for treatment, and higher perceived helpfulness of the treatment.

Conclusion

Belief in helpfulness of treatment is associated with higher parental perceived adherence to treatment.     

575名新疆汉族青年学生手长、掌长与身高的关系

目的：研究新疆汉族青年手长、掌长与身高的关系，为人类学、法医学提供参考，同时为中国人体质调查积累资料。方法：应用Ｈｒｄｌｉｃａｓ标准和我国普遍采用的方法测量了５７５名（男２７０名，女３０５名）１８～２４岁汉族青年学生的手长、掌长和身高，并将原始数据进行医学统计学处理。结果：按年龄组和性别组计算出各组手长、掌长和身高的均值，身高与手长、掌长的比值。并提出由手长、掌长推算身高的简单公式（身高＝手长×比值，身高＝掌长×比值）和回归方程（身高＝回归系数×手长＋截距，身高＝回归系数×掌长＋截距）。结论：手长、掌长和身高各组性差有显著性（Ｐ＜０．０１）；由手长、掌长推算身高的简单公式和回归方程成立（Ｐ＜０．０５）。     

损毁双侧蓝斑区引起血尿的实验研究

用电解损毁方法损毁Ｗｉｓｔａｒ成年雄性大鼠的双侧蓝斑区。结果出现膀胱扩张、尿液潴留和血尿等，膀胱和输尿管尿液有大量红细胞和血红蛋白，膀胱粘膜和肾脏充血．结果表明：损毁双侧蓝斑区后不仅有膀胱出血，还有肾脏出血。这个实验可用作研究“特发性”血尿的动物模型。