Nonlinear optical microscopy is a novel tool for the analysis of cutaneous alterations in pseudoxanthoma elasticum

Lasers in Medical Science - Pseudoxanthoma elasticum (PXE, OMIM 264800) is a rare autosomal recessive disorder with ectopic mineralization and fragmentation of elastin fibers. It is caused by...     

Occurrence of inadequate ACL healing after Dynamic Intraligamentary Stabilization and functional outcome—a multicentre case series

Introduction

Dynamic Intraligamentary Stabilization (DIS) is a technique for preservation, anatomical repair and stabilization of a freshly injured anterior cruciate ligament (ACL). The main purpose of this study was to evaluate the short-term re-operation rate when compared to traditional autograft reconstruction.

Methods

Four, from the developer independent, centres enrolled patients that underwent ACL repair by DIS, according to the specific indications given by MRI imaging at a minimum follow-up of 12 months. The re-operation rate was recorded as primary outcome. Secondary outcome measures were the postoperative antero-posterior knee laxity (using a portable Rolimeter®), as well as the Tegner, Lysholm and IKDC Scores.

Results

A total of 105 patients were investigated with a median follow-up of 21 months. Thirteen patients were lost to follow-up. Of the remaining 92 patients 15 (16.3%) had insufficient functional stability and required subsequent ACL reconstruction. These patients were excluded from further analysis, leaving 77 consecutive patients for a 12 months follow-up. The median age at time of surgery was 30 years for that group. At time of follow-up a median antero-posterior translation difference of 2 mm was measured. None of these patients reported subjective insufficiency (giving way), but in 14 patients (18.2%), the difference of antero-posterior translation was more than 3 mm. We found a median Tegner Score of 5.5, a median Lysholm Score of 95.0 and a median IKDC Score of 89.4.

Conclusion

The main finding of this multicentre study is a relevant re-operation rate of 16.3%. Another 18.2% showed objective antero-posterior laxity (≥ 3 mm) during testing raising the suspicion of postoperative non-healing. The failure rate of DIS in this study is higher than for reconstruction with an autologous tendon graft. However, our successfully treated patients had a good clinical and functional outcome based on antero-posterior knee laxity and clinical scores, comparable to patients treated by autograft reconstruction.

     

Value of upper gastrointestinal endoscopy for gastric cancer surveillance in patients with Lynch syndrome

In our study, we evaluated the effectiveness of upper gastrointestinal (GI) endoscopy as an instrument for early gastric cancer (GC) detection in Lynch syndrome (LS) patients by analyzing data from the registry of the German Consortium for Familial Intestinal Cancer. In a prospective, multicenter cohort study, 1128 out of 2009 registered individuals with confirmed LS underwent 5176 upper GI endoscopies. Compliance was good since 77.6% of upper GI endoscopies were completed within the recommended interval of 1 to 3 years. Forty‐nine GC events were observed in 47 patients. MLH1 (n = 21) and MSH2 (n = 24) mutations were the most prevalent. GCs in patients undergoing regular surveillance were diagnosed significantly more often in an early‐stage disease (UICC I) than GCs detected through symptoms (83% vs 25%; P = .0231). Thirty‐two (68%) patients had a negative family history of GC. The median age at diagnosis was 51 years (range 28‐66). Of all GC patients, 13 were diagnosed at an age younger than 45. Our study supports the recommendation of regular upper GI endoscopy surveillance for LS patients beginning no later than at the age of 30.     

Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) with multiple vascular complications misdiagnosed as Dubowitz syndrome

To date, the genetic basis of Dubowitz syndrome (short stature, microcephaly, facial abnormalities, eczema) is unknown and vascular complications are not known to be associated with this syndrome. In microcephalic osteodysplastic primordial dwarfism type II (MOPD II; disproportionate short statue, microcephaly, facial abnormalities), however, cerebral aneurysms and other vascular abnormalities are frequent complications. MOPD II is a genetic disorder caused by mutations in the pericentrin (PCNT) gene (21q22). We report on a patient who came to our attention as a 22-year-old with subarachnoid bleeding due to a ruptured cranial aneurysm. Until then, the patient was thought and published to have Dubowitz syndrome; previously, he was treated with coronary bypass surgery for extensive coronary angiopathy. Consecutive genetic testing revealed MOPD II. After clinical stabilization, the patient was discharged to a specialized rehabilitation center where he died due to re-rupture of a cranial aneurysm. Conclusion: In patients with short stature—especially when clinical features are accompanied by vascular complications—MOPD II should be considered as a differential diagnosis leading to consecutive genetic testing. After detection of mutations in the PCNT gene, a full vascular status including cerebral imaging and cardiac evaluation needs to be determined in order to analyze vascular abnormalities and initiate prophylactic treatment.     

Tooth Loss,Periodontitis, and Statins in a Population‐Based Follow‐Up Study

Background: Statins, frequently prescribed in lipid‐lowering therapies, seem to have additional beneficial effects on periodontitis and tooth loss. If this is true, then chronic treatment with statins should also result in diminished tooth loss as a long‐term response. Methods: A 5‐year population‐based follow‐up study of tooth loss was performed comparing participants treated with statins (n = 134) with those not on the drugs (Study of Health in Pomerania). Negative binomial regression models were used to analyze the count variable of the outcome, including risk factors for tooth loss and measures of cholesterol metabolism. Results: When adjusted for age and sex, statins were associated with reduced tooth loss during the follow‐up period (incidence risk ratio [IRR] = 0.70, 95% confidence interval [CI] = 0.50 to 0.99, P = 0.04). When additionally adjusted for risk factors of periodontal breakdown, IRR was 0.72 (95% CI = 0.52 to 1.01). There was significant interaction with low‐density lipoprotein cholesterol (LDL‐c) at baseline. After stratification by LDL‐c, statins were associated with reduced tooth loss, resulting in IRR = 0.89 (95% CI = 0.44 to 1.83) and 0.64 (95% CI = 0.43 to 0.95), P = 0.03, at LDL‐c concentrations ≤100 mg/dL and >100 mg/dL (2.58 mmol/L), respectively. The data also showed reduced tooth loss associated with the 5‐year reduction in LDL‐c levels on a mmol/L basis and independently of statins (IRR = 0.87, 95% CI = 0.80 to 0.96, P = 0.004). Conclusion: Long‐term treatment with systemically administered statins may have the beneficial effect of protecting against tooth loss.     

Role of 16-multidetector computed tomography in the assessment of coronary artery stenoses: A prospective study of consecutive patients

BACKGROUND: Recent studies have demonstrated a high sensitivity (S) of 16-multidetector computed tomography (16-MDCT) for the detection of significant coronary artery stenoses. Whether these results are applicable to clinical practice is unclear. Therefore, the aim of the present study was to compare 16-MDCT angiography with conventional coronary angiography (CCA) for the detection of significant coronary artery stenoses in a consecutive series of patients. METHOD: A total of 93 consecutive patients (mean [+/- SD] age 59+/-9 years), in whom CCA was performed for stable angina pectoris, underwent 16-MDCT angiography (16x0.75 mm, table feed 6.5 mm/s, rotation time 0.42 s; Sensation 16, Siemens Medical Solutions, Germany) the day before performing CCA. Patients with diabetes mellitus, serum creatinine level higher than 132.6 mumol/L and/or acute coronary syndromes were excluded. Two observers blinded to CCA results evaluated MDCT angiograms according to standard criteria. Segment-based (13 segments per patient) and patient-based (at least one stenosis greater than 50% lumen diameter reduction) analyses were performed. RESULTS: A total of 1209 segments were analyzed. Of these segments, 173 (14%) were excluded due to poor image quality or massive calcification. In 86 segments, CCA revealed significant coronary artery stenosis (greater than 50% diameter reduction). However, 16-MDCT detected only 47 of these, resulting in a S of 55% and a specificity (SP) of 97% (positive predictive value 64%; negative predictive value 96%). On a patient-based analysis, the S increased to 89%, whereas the SP still remained high (87%). CONCLUSION: In this relatively large consecutive cohort, S for the detection of significant coronary artery stenoses was moderate on a segment-based analysis but increased on a patient-based analysis using 16-MDCT. In contrast, SP was high in both analyses, supporting the use of 16-MDCT for the exclusion of significant coronary artery stenoses. Further improvement of spatial and temporal resolution in MDCT technology may lead to a lower exclusion rate and higher S.     

A RABBIT FAMILY OF RESTRICTED HIGH RESPONDERS TO THE STREPTOCOCCAL GROUP A-VARIANT POLYSACCHARIDE : SELECTIVE BREEDING NARROWS THE ISOELECTRIC FOCUSING SPECTRA OF DOMINANT CLONES

Immunization of rabbits from a closed colony with streptococcal Group A-variant vaccines identified about two-thirds of them as low and heterogeneous responders. One-third of the rabbits showed a restriction of the response independent from the magnitude. Selective breeding from one monoclonal high-responder male and two restricted high-responder female rabbits succeeded in segregation of high-responder progeny after two generations. Their antibody levels were on the average 2.5 times higher than those of the random group of rabbits and a small group of low-responder offspring. Immunization of 13 offspring originating from rabbits bred for restricted high response to the streptococcal Group C polysaccharide revealed that 11 progeny were restricted high responders and 2 progeny monoclonal high responders. This finding suggests that high responsiveness to the Groups A-variant and C polysaccharides is inherited as genetically linked traits. Selective breeding combinations between restricted and monoclonal high-responder rabbits by brother-sister matings succeeded in narrowing the isoelectric focusing spectra of Group A-variant-specific antibodies in the offspring. It furthermore revealed a preferential expression of monoclonal antibodies after three generations with a similar net charge as those identified first in the original monoclonal paternal parent. These data suggest that similar copies of structural genes for the variable regions are transmitted from the parent to the progeny.     

Comparing emergency medical service systems--a project of the European Emergency Data (EED) Project

Aim

The aim of this prospective study was the comparison of four emergency medical service (EMS) systems—emergency physician (EP) and paramedic (PM) based—and the impact of advanced live support (ALS) on patients status in preclinical care.

Methods

The EMS systems of Bonn (GER, EP), Cantabria (ESP, EP), Coventry (UK, PM) and Richmond (US, PM) were analysed in relation to quality of structure, process and performance when first diagnosis on scene was cardiac arrest (OHCA), chest pain or dyspnoea. Data were collected prospectively between 01.01.2001 and 31.12.2004 for at least 12 month.

Results

Over all 6277 patients were included in this study. The rate of drug therapy was highest in the EP-based systems Bonn and Cantabria. Pain relief was more effective in Bonn in patients with severe chest pain. In the group of patients with chest pain and tachycardia ≥120 beats/min, the heart rate was reduced most effective by the EP-systems. In patients with dyspnoea and S_pO₂ < 90% the improvement of oxygen saturation was most effective in Bonn and Richmond. After OHCA significant more patients reached the hospital alive in EMS systems with EPs than in the paramedic staffed (Bonn = 35.6%, Cantabria = 30.1%; Coventry = 11.9%, Richmond = 9.2%). The introduction of a Load Distributing Band chest compression device in Richmond improved admittance rate after OHCA (21.7%) but did not reach the survival rate of the Bonn EMS system.

Conclusions

Higher qualification and greater training and experience of ALS unit personnel increased survival after OHCA and improved patient's status with cardiac chest pain and respiratory failure.     

Working memory maintenance of grasp-target information in the human posterior parietal cortex

Event-related functional magnetic resonance imaging was applied to identify cortical areas involved in maintaining target information in working memory used for an upcoming grasping action. Participants had to grasp with their thumb and index finger of the dominant right hand three-dimensional objects of different size and orientation. Reaching-to-grasp movements were performed without visual feedback either immediately after object presentation or after a variable delay of 2-12 s. The right inferior parietal cortex demonstrated sustained neural activity throughout the delay, which overlapped with activity observed during encoding of the grasp target. Immediate and delayed grasping activated similar motor-related brain areas and showed no differential activity. The results suggest that the right inferior parietal cortex plays an important functional role in working memory maintenance of grasp-related information. Moreover, our findings confirm the assumption that brain areas engaged in maintaining information are also involved in encoding the same information, and thus extend previous findings on working memory function of the posterior parietal cortex in saccadic behavior to reach-to-grasp movements.