Recurrent dislocation of the patella and the Goldthwait operation

In this study 17 patients with recurrent dislocation of the patella were followed up 10 years after their Goldthwait operation. The subjective and clinical findings were excellent or good in 70%. X-radiographs indicated osteoarthritis of the femoropatellar joint in 60%. Concerning the aetiopathological factors, we found an increased external torsion of the afflicted extremity (measured by computed tomography).     

DOES LIVER DYSFUNCTION EXPLAIN NEUROPSYCHOLOGICAL STATUS IN RECENTLY DETOXIFIED ALCOHOL-DEPENDENT CLIENTS?

In the search for explanation of persistent cognitive impairmentassociated with alcohol dependence, the possible role of liverdisease has aroused considerable interest. However, review ofthe relevant literature provides only ambiguous support forany general relationship between neuropsycho-logical statusand laboratory tests of liver function. We tested the generalhypothesis, and also two specific hypotheses relating particularliver function parameters (     

Adverse reactions following pulsed tunable dye laser treatmentof port wine stains in 701 patients

The pulsed tunable dye laser (PTDL) is generally considered to have a very low incidence of adverse effects, allowing it to become the treatment of choice for the majority of port wine stains (PWS). The low incidence of adverse effects has led to difficulties in determining the true incidence and type of adverse effect seen with this laser. We therefore undertook a retrospective study of 701 patients with PWS, who received 3877 full treatments to determine the incidence and type of adverse effects seen following treatment with the PTDL. Blistering and crusting were seen in 5·9% and 0·7% of patients, respectively, but were transient events which usually healed without permanent sequelae. Hyperpigmentation was the most frequently observed adverse effect seen in 9·1% of patients but generally showed gradual resolution over 6–12 months. Hypopigmentation was infrequent, seen in 1.4% of patients. The most significant adverse effects were atrophic and hypertrophic scarring seen in 4·3% and 0·7% of patients, respectively. Our observations show that there is a small but definite risk of atrophic scarring with a predisposition for younger patients. Hypertrophic scarring can occur albeit rarely and there may be a predisposition towards the neck. In most cases test areas were not predictive of scarring. This underlines the need for a full discussion of scarring risk in patients with PWS undergoing treatment with the PTDL.     

Bullous pemphigoid in association with cutaneous lesions specific to a myelodysplastic syndrome

Specific cutaneous lesions are a rare occurrence in myelodysplastic syndromes (MDS). The concurrent association of blistering skin lesions similar to those in bullous pemphigoid (BP), even though a rare event, suggests that BP may be a paraneoplastic syndrome. We report an 86-year-old man who had a refractory anaemia with excess bone marrow blasts in transformation, who developed a generalized pruritic blistering eruption. Immunohistopathological tests showed subepidermal blisters with linear deposits of IgG and C3 along the basement membrane zone of the epidermis surrounding a tumoral dermal infiltrate of CD13+ and CD15+ cells. Immunoblotting studies using epidermal extracts revealed circulating IgG antibodies against three protein bands: a 210–215 kDa band. a 180kDa band which co-migrated with the BP 180 antigen, and a 190kDa band. The tumour infiltrate may have revealed antigenic determinants which led to the onset of BP. The concept of paraneoplastic pemphigoid remains to be either confirmed or invalidated by further epidemiological studies.     

Bone mineral density in patients with atopic dermatitis

With the aim of evaluating the systemic effect of glucocorticoids (GCs), we measured bone mineral density (BMD) in 29 adult patients with chronic atopic dermatitis. BMD was measured in the lumbar spine and in the left femoral neck using dual–energy X–ray absorptiometry (DEXA). In the right calcaneus. BMD was measured using broad–spectrum ultrasound attenuation (BUA). For BMD, the patients with dermatitis did not differ from healthy controls. We did not find any statistically significant correlation between BMD and single risk factors, such as the barrier function of the skin (P = 0.08), the duration of the dermatitis (P = 0.58), and the use of oral GCs (P = 0.27) and potent topical GCs (P = 0.10). However, selected patients with severe disease, needing topical GCs of higher potency than hydrocortisone (HC), had lower values for lumbar BMD than the patients who had not used these preparations (–1.0 vs. +0.1 SD; P = 0.026). The lower lumbar BMD in this group could be explained by a long-term systemic effect of GCs.     

Effect of UVB 311 nm irradiation on normal human skin

Ultraviolet radiation B (UVB) on the skin induces erythema, inflammation and modifications of the immune system. These changes have been reported after excessive short-term or long-term exposure to broad spectrum UVB. In this study, we examined the effects of local repetitive UVB irradiation of 311 nm wavelength on the skin of seven young volunteers. Skin biopsies were taken before and after UVB irradiation, and we immunohistochemically analyzed the expression of CD1a and HLA-DR antigens of Langerhans cells (LC), the possible infiltration of dermis/epidermis by CD11b macrophages, the modifications or the induction of intercellular adhesion molecule-1 (ICAM-1), E-selectin and vascular cell adhesion molecule-1 (VCAM-1) involved in the binding of leukocytes to the endothelial surface and the development of perivascular infiltrates of LFA-1⁺ mononuclear cells. We also determined the expression of substance P receptors (SPR) using biotinylated substance P (SPB). Exposure of UVB 311 nm induced a drastic reduction of CD1a⁺ cells and a moderate increase of HLA-DR⁺ dendritic cells in the epidermis without infiltration by CD11b macrophages. An increase of the binding of SPB to upper layer epidermal cells was noted in five of seven biopsies. In the dermis, vessel-associated ICAM-1 expression increased and an induction of E-selectin occurred on nearly 20 to 40% of endothelial cells, but VCAM-1 expression remained undetectable. The percentage of LFA-1⁺ cells did not change significantly after irradiation. These observations may be compatible with a selective role of UVB 311 nm on the skin immune response.     

Detection of neutral protease (Periocheck) and BANA hydrolase (Perioscan) compared with traditional clinical methods of diagnosis and monitoring of chronic inflammatory periodontal disease

Abstract Perioscan requires a plaque sample to detect the presence of enzymes capable of degrading N-benzoyl-DL-arginine-2-naphthylamide (BANA) from relatively few anaerobic periodontal pathogens. Periocheck assays the presence of neutral proteases in crevicular fluid. The aim of this study was to compare these test kits with traditional clinical methods of detecting periodontal disease and to monitor the ability of the kits to reflect the response to initial therapy. 19 patients with moderately severe chronic periodontitis were seen before and after a course of oral hygiene and root instrumentation consisting of 4 appointments. Clinical measurements and test assays were collected at 5 diseased sites and 2 healthy sites in each subject. Complete data from 125 sites were available for statistical analysis. At baseline Periocheck had a sensitivity of 88% and a specificity of 61% whereas Perioscan had a sensitivity of 99% and a specificity of 55%, when related to the clinical diagnosis. A composite clinical assessment, based on improvement or deterioration of one whole unit change of the subjective clinical indices and 2mm changes or greater in probing depth or probing attachment level, revealed 75 sites which improved following treatment, whereas 45 sites did not change and 5 sites deteriorated. The probability that the tests agreed with the clinical outcome after treatment, was calculated as 50.4% for Periocheck and 52% for Perioscan. The diagnostic kits did not reliably reflect the clinical assessment of periodontal disease in the cross sectional study, or the outcome following treatment.     

Periapical radiographic techniques during endodontic diagnosis and treatment

The objective of this article is to describe various radiographic projections which can be used during endodontic therapy. Changes to the angulation of the X-ray beam in relation to the teeth and film can help diagnosis and treatment by producing images which provide additional information not always visible on radiographs taken with standard angulations. For example, changes in angulation can be useful to determine the number and curvature of roots and canals, to identify superimposed roots and to distinguish between anatomical landmarks and apical pathology. Although use of such techniques increases the diagnostic yield of films, it must be appreciated that such views lead to images that are less distinct because of inherent image distortion. Nevertheless, use of the various techniques during endodontics can provide substantial benefit for clinicians in their daily practice.     

心肌造影超声心动图评估心肌缺血程度的实验研究

本实验制作不同程度心肌缺血的动物模型，以声处理５％人血白蛋白为超声造影剂进行心肌灌注造影，探讨ＭＣＥ时间－强度曲线各指标与心肌缺血程度间的关系。结果表明：心肌显影的峰值强度和曲线下面积均与缺血程度呈显著负相关（相关系数分别为ｒ＝－０．９８，Ｐ值＜０．００５；ｒ＝－０．９４，Ｐ值＜０．０５），且能区分轻度、中度和重度三种程度的心肌缺血；而三项时间指标与心肌缺血程度间未发现显著性差异。本实验的初步结果表明，ＭＣＥ是一项活体评估局部心肌血流灌注的有效方法。     

Missense mutations in codon 225 of ornithine transcarbamylase (OTC) result in decreased amounts of OTC protein: A hypothesis on the molecular mechanism of the OTC deficiency

Mutations P225L and P225R were identified in codon 225 of the gene for ornithine transcarbamylase (OTC) in two patients with the neonatal form of OTC deficiency. The mutations occur at a CpG dinucleotide and eliminate a unique MspI restriction site in exon 7 of the OTC gene. They do not alter existing splice sites or create new sites, as judged from the nucleotide sequence. Both mutations are associated with undetectable levels of OTC antigen in liver homogenates, and with either complete lack of OTC activity (P225R mutation) or very small residual activity (0.15% of normal in the P225L mutation). The residual activity observed with P225L exhibits normal pH dependence, little or no increases in the Km values for ornithine and carbamoyl phosphate and normal stability at either 37°C or, in the presence of 0.66 mol/L urea, at 0°C. The latter conditions were used to examine whether the P225L mutation favours dissociation of the active OTC trimer. Given the normal stability and lack of tendency to dissociation of the mutant enzyme, it appears likely that the dramatic reduction in the level of OTC protein is due to inefficient conversion of the mutant OTC precursor polypeptide (pOTC) into the correctly localized, appropriately folded, mature enzyme trimer, suggesting degradation of pOTC in transit to the mitochondria.