Complex infantile and congenital hip dislocation: assessment with MR imaging

Twenty-five magnetic resonance hip studies were performed on 19 infants with congenital hip dislocation. These patients had a poor initial treatment response, a teratologic dislocation, or a late presentation. Detailed images of single hips obtained with small surface coils resulted in excellent visualization of all the clinically important soft-tissue and cartilaginous structures of the hip. No other imaging modality demonstrates all of these structures simultaneously.     

Attentional processing in common whiplash syndrome before and with immobilisation of the cervical spine

Summary The study focused on attentional processes in patients who had suffered common whiplash injury and who were investigated before and with immobilisation of the cervical spine. Patients demonstrated considerable subjective relief and marked improvement in complex attentional processing with the immobilisation. This improvement cannot be explained by the practice effect as shown by the results in a control group. The norepinephrine system is discussed as a possible neurophysiological basis for the observed effects.     

ANEURYSMAL BONE CYST IN THE MANDIBLE (A Case Report)

Aneurysmal bone cyst is commonly seen in the long bones and vertebrae and is rare in the jaws. Its association with other lesions of the bone has been stressed by many workers. Because of its variable radiological appearance, diagnosis of the lesion is established by histopathological examination. A case of aneurysmal bone cyst occurring in the mandible is reported.KEY WORDS: Aneurysmal bone cyst, Mandible     

Attentional processing in cervical spine syndromes

Fifty-four patients suffering from "common whiplash" were compared with 28 patients complaining of cervical spine syndrome caused by rheumatism (Barré-Lieou syndrome). All patients underwent clinical interview and formal testing. Formal testing included personality profile, self ratings of cognitive impairment and well-being, and tasks on divided attention and speed of information processing. In the "common whiplash" group higher relative incidences of adjustment disorder were found. In both groups scoring for divided attention was low. With regard to self-ratings, the group suffering from Barré-Lieou syndrome hardly indicated any problems while the "common whiplash" group showed significant impairment. This difference of self-ratings was assumed to reflect the different modes of development of the investigated syndromes: in the group with cervical syndrome due to rheumatism the condition gradually developed giving the patient the possibility to adapt, while in the "common whiplash" group cervical pathology emerged abruptly by impact injury. Adaptation in the latter group was inadequate which in particular explains the appearance of adjustment disorders amongst "common whiplash" patients. Results indicated that headache due to cervical pathology is likely to be responsible for impaired attentional functioning of "common whiplash" patients.     

Evidence for spinal cord hypersensitivity in chronic pain after whiplash injury and in fibromyalgia

Patients with chronic pain after whiplash injury and fibromyalgia patients display exaggerated pain after sensory stimulation. Because evident tissue damage is usually lacking, this exaggerated pain perception could be explained by hyperexcitability of the central nervous system. The nociceptive withdrawal reflex (a spinal reflex) may be used to study the excitability state of spinal cord neurons. We tested the hypothesis that patients with chronic whiplash pain and fibromyalgia display facilitated withdrawal reflex and therefore spinal cord hypersensitivity. Three groups were studied: whiplash (n=27), fibromyalgia (n=22) and healthy controls (n=29). Two types of transcutaneous electrical stimulation of the sural nerve were applied: single stimulus and five repeated stimuli at 2 Hz. Electromyography was recorded from the biceps femoris muscle. The main outcome measurement was the minimum current intensity eliciting a spinal reflex (reflex threshold). Reflex thresholds were significantly lower in the whiplash compared with the control group, after both single (P=0.024) and repeated (P=0.035) stimulation. The same was observed for the fibromyalgia group, after both stimulation modalities (P=0.001 and 0.046, respectively). We provide evidence for spinal cord hyperexcitability in patients with chronic pain after whiplash injury and in fibromyalgia patients. This can cause exaggerated pain following low intensity nociceptive or innocuous peripheral stimulation. Spinal hypersensitivity may explain, at least in part, pain in the absence of detectable tissue damage.     

Central hypersensitivity in chronic pain after whiplash injury.

OBJECTIVE: The mechanisms underlying chronic pain after whiplash injury are usually unclear. Injuries may cause sensitization of spinal cord neurons in animals (central hypersensitivity), which results in increased responsiveness to peripheral stimuli. In humans, the responsiveness of the central nervous system to peripheral stimulation may be explored by applying sensory tests to healthy tissues. The hypotheses of this study were: (1) chronic whiplash pain is associated with central hypersensitivity; (2) central hypersensitivity is maintained by nociception arising from the painful or tender muscles in the neck. DESIGN: Comparison of patients with healthy controls. SETTING: Pain clinic and laboratory for pain research, university hospital. PATIENTS: Fourteen patients with chronic neck pain after whiplash injury (car accident) and 14 healthy volunteers. OUTCOME MEASURES: Pain thresholds to: single electrical stimulus (intramuscular), repeated electrical stimulation (intramuscular and transcutaneous), and heat (transcutaneous). Each threshold was measured at neck and lower limb, before and after local anesthesia of the painful and tender muscles of the neck. RESULTS: The whiplash group had significantly lower pain thresholds for all tests. except heat, at both neck and lower limb. Local anesthesia of the painful and tender points affected neither intensity of neck pain nor pain thresholds. CONCLUSIONS: The authors found a hypersensitivity to peripheral stimulation in whiplash patients. Hypersensitivity was observed after cutaneous and muscular stimulation, at both neck and lower limb. Because hypersensitivity was observed in healthy tissues, it resulted from alterations in the central processing of sensory stimuli (central hypersensitivity). Central hypersensitivity was not dependent on a nociceptive input arising from the painful and tender muscles.     

Prenatal growth restriction,retinal dystrophy,diabetes insipidus and white matter disease: expanding the spectrum of PRPS1-related disorders

PRPS1 codes for the enzyme phosphoribosyl pyrophosphate synthetase-1 (PRS-1). The spectrum of PRPS1-related disorders associated with reduced activity includes Arts syndrome, Charcot–Marie–Tooth disease-5 (CMTX5) and X-linked non-syndromic sensorineural deafness (DFN2). We describe a novel phenotype associated with decreased PRS-1 function in two affected male siblings. Using whole exome and Sanger sequencing techniques, we identified a novel missense mutation in PRPS1. The clinical phenotype in our patients is characterized by high prenatal maternal α-fetoprotein, intrauterine growth restriction, dysmorphic facial features, severe intellectual disability and spastic quadraparesis. Additional phenotypic features include macular coloboma-like lesions with retinal dystrophy, severe short stature and diabetes insipidus. Exome sequencing of the two affected male siblings identified a shared putative pathogenic mutation c.586C>T p.(Arg196Trp) in the PRPS1 gene that was maternally inherited. Follow-up testing showed normal levels of hypoxanthine in urine samples and uric acid levels in blood serum. The PRS activity was significantly reduced in erythrocytes of the two patients. Nucleotide analysis in erythrocytes revealed abnormally low guanosine triphosphate and guanosine diphosphate. This presentation is the most severe form of PRPS1-deficiency syndrome described to date and expands the spectrum of PRPS1-related disorders.     

细胞凋亡与肝脏疾病

细胞凋亡(apoptosis,APO)是指在一定的生理和病理情况下,机体为维护内环境的稳定,通过基因调控而使细胞自动消亡的过程.英国Kerr教授1972年首先提出凋亡的概念,他主要根据形态学上的不同将细胞凋亡与坏死相区分,并指出前者是一种主动的、程序性的、细胞固有的过程许多文献又称凋亡为程序性细胞死亡(programmed cell death,PCD),而最新观念认为APO是形态学上的概念,PCD是发生机制上的概念,不是所有PCD都表现为APO,因而可以认为APO是PCD的一种形态学表现[1].1980年以来,细胞凋亡现象受到了广泛重视,有关的研究工作取得了重要进展,并成为医学生物学各学科所共同关注的极为活跃的研究领域[2-19].