What’s new in the aetiopathogenesis of vasculitis?

The cause of the majority of childhood vasculitides is unknown although it is likely that a complex interaction between environmental factors and inherited host responses trigger the disease and determine the vasculitis phenotype. Epidemiological clues continue to implicate infectious triggers in Kawasaki syndrome (KS) and Henoch Schőnlein purpura (HSP). Several genetic polymorphisms have now been described in KS and HSP which predispose to disease or predict disease severity. Anti-neutrophil cytoplasmic antibodies (ANCA) are now known to be directly involved in the pathogenesis of vascular injury in ANCA-associated vasculitides, although why some individuals develop ANCA in the first instance is not yet understood. Endothelial injury and repair are active areas of research in vasculitis. It is now possible to track endothelial injury non-invasively in children with vasculitis using surrogate markers of endothelial injury. The vasculogenic pathways involved in vascular repair following vasculitis, including endothelial progenitor cells, are beginning to be studied. It is anticipated that an improved understanding of the aetiopathogenesis of vasculitis in the young will ultimately shape future novel diagnostic and therapeutic approaches and will help us predict which children may develop premature arteriosclerosis in later life.     

Parent Abuse: A Review

A recent focus of research and clinical practice has been on the issue of abuse of parents by their children (parent abuse). This paper reviews the literature on this phenomenon. While parent abuse falls under the umbrella of family violence, it appears to be qualitatively different from other forms of intra-family abuse. Research has primarily focused on prevalence rates and the characteristics of perpetrators and victims. While various factors such as gender, age, emotional attachment to parents, race/ethnicity, socioeconomic status, family stress and parenting style and structure have been associated with parent abuse, findings are equivocal. Etiological models are general and untested, and treatment approaches lack empirical support. It is concluded that more rigorous and extensive research is required in order to provide a deeper understanding of this complex issue, and to inform treatment approaches.     

Budd-Chiari综合征中下腔静脉阻塞膜与肝右静脉的位置关系研究

目的分析下腔静脉阻塞膜与肝右静脉的位置关系,探讨Budd-Chiari综合征（BCS）可能的病因学。方法 35例下腔静脉IVC膜性阻塞性病变患者,DSA观察下腔静脉CIVC阻塞膜与肝静脉的位置关系。结果膜位于肝右静脉以上者32例,肝右静脉以下者3例。膜位于肝右静脉与肝左、中静脉之间者3例,位于三支HV以上者4例。结论某些低毒性物质的作用和IVC壁的损伤可能为BCS发病的始动因素。     

Hippocampal sclerosis in children with lesional epilepsy is influenced by age at seizure onset

PURPOSE: Hippocampal sclerosis (HS) is the most common lesion underlying drug-resistant temporal lobe epilepsy. Whether HS is a developmental or acquired pathology remains unclear. Whereas HS has been causally linked to prolonged febrile convulsions in childhood, evidence also exists that it may coexist with extrahippocampal abnormalities, the concept of "dual pathology." The aims of this study were to address whether hippocampal abnormality consistent with HS (a) occurs in children with lesional extrahippocampal epilepsy, (b) is more commonly seen in association with developmental rather than acquired extrahippocampal pathologies, and (c) whether any effect of age at seizure onset is found on the occurrence of HS in lesional extrahippocampal epilepsy. METHODS: Clinical and histopathologic data of patients having resective surgery for extrahippocampal epilepsy that included the hippocampus were investigated. RESULTS: Twenty-nine children were retrospectively included in this study, and 21 (72%) of 29 were found to have a hippocampal abnormality consistent with HS. No relation was noted between developmental or acquired extrahippocampal pathologies and the presence of hippocampal abnormality. Children with normal hippocampi on visual histologic assessment had a significantly younger age at seizure onset (p < 0.001). Duration of epilepsy was not correlated with the presence of hippocampal abnormality. CONCLUSIONS: Hippocampal abnormalities are seen in similar proportions with both acquired and developmental extra-hippocampal pathologies, suggesting that these abnormalities are the result of seizures from the focus that is remote from the hippocampus. In addition, children who have their initial seizure at an early age are less likely to develop seizure-induced hippocampal injury.     

Rectal prolapse

INTRODUCTION: Rectal prolapse, or procidentia, is defined as a protrusion of the rectum beyond the anus. It commonly occurs at the extremes of age. Rectal prolapse frequently coexists with other pelvic floor disorders, and patients have symptoms associated with combined rectal and genital prolapse. Few patients, a lack of randomized trials and difficulties in the interpretation of studies of anorectal physiology have made the understanding of this disorder difficult. METHODS OF TREATMENT: Surgical management is aimed at restoring physiology by correcting the prolapse and improving continence and constipation, whereas in patients with concurrent genital and rectal prolapse, an interdisciplinary surgical approach is required. Operation should be reserved for those patients in whom medical treatment has failed, and it may be expected to relieve symptoms. Numerous surgical procedures have been suggested to treat rectal prolapse. They are generally classified as abdominal or perineal according to the route of access. However, the controversy as to which operation is appropriate cannot be answered definitively, as the extent of a standardized diagnostic assessment and the types of surgical procedures have not been identified in published series. LITERATURE REVIEW: This review encompasses rectal prolapse, including aetiology, symptoms and treatment. The English-language literature about rectal prolapse was identified using Medline, and additional cited works not detected in the initial search were obtained. Articles reporting on prospective and retrospective comparisons and case reports were included.     

STUDY ON THE PRECANCEROUS ESOPHAGEAL LESIONSOF RHESUS MONKEYS FROM TAIHANG AREAOF HIGH MORBIDITY OF ESOPHAGEAL

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The jigsaw sign. A reliable indicator of congenital aetiology in os odontoideum

There is evidence in the literature for both a congenital and a post-traumatic aetiology for os odontoideum. In no series published to date has CT been used to aid in the diagnosis. This is a prospective study of the history of trauma and presence of diagnostic features on CT of 18 consecutive cases with os odontoideum. Our objective was to derive clinically useful radiological features enabling accurate differentiation between congenital and post-traumatic aetiologies. A mid-sagittal CT reconstruction of the atlanto-dens joint was obtained. Hypertrophy of the anterior arch of the atlas was quantified by measurement of the arch-peg-area ratio. The presence of dysplastic features (a positive jigsaw sign) of the atlanto-axial joint were noted. These included narrowing of the cartilage space and interdigitation of the two joint surfaces. A history of a potential traumatic aetiology was only obtained in one of the 18 (6%) in our series. A significant elevation of the arch–peg ratio was found when comparing this series to 85 controls. And a positive jigsaw sign was observed in 75% of cases. These features were not seen in paediatric cases of atlanto-axial instability, including odontoid non-union. In conclusion, an elevated arch–peg ratio and the presence of a jigsaw sign are sensitive and specific diagnostic criteria for os odontoideum. This series supports a congenital aetiology for this condition.Institution where work carried out: Spine Research Group, School of Mechanical, Manufacturing and Medical Engineering, Queensland University of Technology, Mater Misericordiae Hospitals Brisbane, GPO Box 2434 Brisbane Q4001, Australia