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991.
BACKGROUND: Laparoscopic surgery in patients with Crohn's disease (CD) has been demonstrated to have advantages over a conventional approach in children. The aim of this study was to review the children treated for CD with a laparoscopic approach, to report our indications, the surgical procedure, the complications, and to compare the children with pancolitis or ileocaecal (segmental) Crohn's disease. PATIENTS AND METHOD: We reviewed the files of 11 children treated for CD in a single institution between 1999 and 2004 for a retrospective study of clinical and surgical data. Mann-Whitney U-test was used for statistical analysis of nonparametric data. RESULTS: Eleven children were operated. The average age when initial clinical symptoms became apparent was 12.1 years (range 6.6 - 15), and surgery was performed after an average of 3.4 years of disease (range 1 - 7.6). The surgical indications were stenosis in 6 cases, failure to thrive in 1 case (segmental CD, SCD group) and pancolitis refractory to medical treatment in 4 cases (pancolitis group, PCD group). Mean operative time was 207 minutes (range 140 - 270) for the SCD group and 285 minutes (range 260 - 300) for the PCD group (p < 0.05). Three cases needed a conversion to open surgery (2 in PCD group, one in SCD group), mainly in relation to anastomosis performed with an EEA stapler. The average length of surgical unit stay was 6.5 days (range 4 - 8) for the PCD group and 6.4 days (range 4 - 8) for the SCD group; average follow-up was 16 months (range 3 - 38). Two patients had a relapse of CD (stenosis of the anastomosis in one, skin fistula in the other). CONCLUSION: A laparoscopic approach for ileocolic resection in Crohn's disease is a feasible procedure, even in cases of pancolitis. We recommend an extra-corporeal anastomosis because, in relation to the inflammatory bowel, the mechanical anastomosis is not a safe procedure in cases of pancolitis.  相似文献   
992.
PURPOSE: In real life, divergence is frequently combined with vertical saccades. The purpose of this study was to examine the initiation of vertical and horizontal saccades, pure or combined with divergence. METHODS: We used a gap paradigm to elicit vertical or horizontal saccades (10 degrees), pure or combined with a predictable divergence (10 degrees). Eye movements from 12 subjects were recorded with EyeLink II. RESULTS: The major results were (i) when combined with divergence, the latency of horizontal saccades increased but not the latency of vertical saccades; (ii) for both vertical and horizontal saccades, a tight correlation between the latency of saccade and divergence was found; (iii) when the divergence was anticipated, the saccade was delayed. CONCLUSION: We conclude that the initiation of both components of combined movements is interdependent.  相似文献   
993.
Is this thyroid hyperplasia true carcinoma? In view of the facts that young fish are more affected than old fish, that iodin stops the hyperplasia and causes it to return to the colloid state, that removal of the fish from the ponds to the open brook also affects a cure, that the clinical incidence of tumors is directly related to the water supply, and that the severity of the disease as determined by histological examination is likewise directly related to the water supply, we cannot accept the prevailing opinion that the disease is true cancer. On the other hand, we believe that the disease as studied by us is an extreme illustration of endemic goitre, the end stage of which is cretinism. The opinion that it is cancer is based purely on anatomical studies—the essential fact being that the growing thyroid invades bone, muscle, etc. Such findings in mammalian tissues are strong evidence of malignancy, but this standard cannot be applied to fish thyroids for the reason that any growth of a widely and loosely distributed nonencapsulated gland would give the histological appearance of invasion of the surrounding tissues. The general views as to the cause of the disease may be divided into two groups: (I) infectious and (2) metabolic. Taking up the infectious theory, there are abundant grounds for such a view when one considers the flora of the tank. The very careful histological examinations by Hofer, Bonnet and Pick have yielded uniformly negative results. Direct infection cannot be excluded, but the fact that fish placed in the tail-race recover spontaneously, although living in the theoretically most polluted water, is not in harmony with our present conception of water-borne infection. Taking up the metabolic theory, the three factors of overfeeding, overcrowding and limited proportional water supply together with the many possibilities into which they are divisible seem to us to be in some way, still obscure, directly concerned with the development of the disease. It is not probable that a single substance, whether the result of food decomposition or of excretory products, excites the thyroid to its activity but rather that the thyroid reaction is the result of the activity of a great variety of these products.  相似文献   
994.
995.
996.

Objective

We aimed to compare the prevalence of enthesopathy seen on ultrasonography (US) in spondyloarthritis (SpA) and rheumatoid arthritis (RA) and compared it to healthy controls.

Methods

All included patients with RA (2010 ACR/EULAR criteria) and SpA (ASAS criteria) and healthy controls underwent clinical and US evaluation of enthesis at seven sites (quadriceps, proximal and distal patellar, Achilles and triceps tendons, plantar aponeurosis and lateral epicondyle enthesis). The Glasgow Ultrasound Enthesitis Scoring System (GUESS) and the Madrid Sonographic Enthesitis Index (MASEI) scores were determined by two sonographers blinded to clinical data.

Results

We included 30 patients with RA (mean age: 55.7 ± 14.8 years, mean disease duration 10.5 ± 7.9 years); 41 with SpA (mean age: 45.3 ± 15.4 years, mean disease duration 9.2 ± 8.7 years) and 26 healthy controls (HC) (mean age: 50.4 ± 17.3 years). Patients with SpA and RA had similar prevalence of painful enthesis of examined sites (17% vs. 14%, non-significant [ns]), but more than among in healthy controls (3%, P < 0.05 for RA and SpA comparison). Comparison between SpA and RA patients revealed that at least one US enthesis abnormality was found with similar frequency (46% and 48% sites [ns]) but both rheumatic diseases had higher frequency of US enthesis abnormality than HC (31%, P < 0.05 for RA and SpA comparison). The mean MASEI score was 8.5 ± 7.3 for RA patients, 7.8 ± 6.5 for SpA patients (ns) and 3.4 ± 2.8 for healthy controls (P < 0.05 for RA and SpA comparison). Overall, 6 RA (20%) and 4 SpA (10%) patients had a MASEI score  18 (ns). None of the healthy controls had a MASEI score  18 (P < 0.05 for RA and SpA comparison). The mean GUESS score was 5.8 ± 3.1 and 6.3 ± 3.9 for RA and SpA patients (ns), and 4.0 ± 3.1 for healthy controls (P < 0.01 vs. SpA and < 0.05 vs. RA).

Conclusions

RA and SpA patients did not differ in entheseal abnormalities seen on US. Such US features may have low specificity in inflammatory conditions affecting joints and enthesis such as SpA and RA.  相似文献   
997.

Introduction

Lipin-1 deficiency is a major cause of rhabdomyolysis that are precipitated by febrile illness. The prognosis is poor, with one-third of patients dying from cardiac arrest during a crisis episode. Apart from acute rhabdomyolysis, most patients are healthy, showing normal clinical and cardiac ultrasound parameters.

Patients and methods

We report cardiac and exercise examinations of 8 children carrying two LPIN1 mutations. The examinations were performed outside of a myolysis episode, but one patient presented with fever during one examination.

Results

All but one patient displayed normal resting cardiac function, as determined by echocardiography. One patient exhibited slight left ventricular dysfunction at rest and a lack of increased stroke volume during cycle ramp exercise. During exercise, peripheral muscle adaptation was impaired in 2 patients compared to healthy controls: they presented an abnormal increase in cardiac output relative to oxygen uptake: dQ/dVO2 = 8.2 and 9.5 (> 2DS of controls population). One patient underwent 2 exercise tests; during one test, the patient was febrile, leading to acute rhabdomyolysis in the following hours. He exhibited changes in recovery muscle reoxygenation parameters and an increased dQ/dVO2 during exercise compared with that under normothermia (7.9 vs 6), which did not lead to acute rhabdomyolysis. The four patients assessed by cardiac 1H-magnetic resonance spectroscopy exhibited signs of intracardiac steatosis.

Conclusion

We observed abnormal haemodynamic profiles during exercise in 3/8 patients with lipin-1 deficiency, suggesting impaired muscle oxidative phosphorylation during exercise. Fever appeared to be an aggravating factor. One patient exhibited moderate cardiac dysfunction, which was possibly related to intracardiac stored lipid toxicity.  相似文献   
998.
An extended-spectrum β-lactamase (ESBL)-producing and colistin-resistant Klebsiella pneumoniae clinical isolate was recovered from a patient who was treated with cefotaxime. This isolate harbored a blaCTX-M-15 ESBL gene that was associated with an ISEcp1 insertion sequence. Transposition of that tandem occurred within the chromosomal mgrB gene, leading to inactivation of the mgrB gene and consequently to acquired resistance to colistin. We showed here a coselection of colistin resistance as a result of a broad-spectrum cephalosporin selective pressure.  相似文献   
999.
Bone regeneration involves skeletal stem/progenitor cells (SSPCs) recruited from bone marrow, periosteum, and adjacent skeletal muscle. To achieve bone reconstitution after injury, a coordinated cellular and molecular response is required from these cell populations. Here, we show that SSPCs from periosteum and skeletal muscle are enriched in osteochondral progenitors, and more efficiently contribute to endochondral ossification during fracture repair as compared to bone-marrow stromal cells. Single-cell RNA sequencing (RNAseq) analyses of periosteal cells reveal the cellular heterogeneity of periosteum at steady state and in response to bone fracture. Upon fracture, both periosteal and skeletal muscle SSPCs transition from a stem/progenitor to a fibrogenic state prior to chondrogenesis. This common activation pattern in periosteum and skeletal muscle SSPCs is mediated by bone morphogenetic protein (BMP) signaling. Functionally, Bmpr1a gene inactivation in platelet-derived growth factor receptor alpha (Pdgfra)-derived SSPCs impairs bone healing and decreases SSPC proliferation, migration, and osteochondral differentiation. These results uncover a coordinated molecular program driving SSPC activation in periosteum and skeletal muscle toward endochondral ossification during bone regeneration. © 2022 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).  相似文献   
1000.
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