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991.
992.
A 17-year-old Croatian boy with Nijmegen breakage syndrome (NBS) who developed diffuse large B-cell non-Hodgkin lymphoma is presented. The majority of the patients with this rare autosomal recessive disease are of Slavic origin and, in most of them, the disease is caused by NBS1 mutation 657del5, as was found in our patient. Nijmegen breakage syndrome is characterized by microcephaly, growth retardation, abnormal facial appearance, spontaneous chromosomal rearrangements, immunodeficiency, and a high predisposition to cancer development, predominantly lymphoma. Because of increased sensitivity to radiation therapy and chemotherapy, the treatment of malignancies in patients with NBS can be difficult. To our knowledge, our patient is the first with NBS reported in the literature who was successfully treated for diffuse large B-cell lymphoma with the anti-CD20 monoclonal antibody rituximab in addition to a modified dose of CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) chemotherapy. He has been in complete remission for 3 years after finishing the treatment.  相似文献   
993.
This study aimed at identifying factors related to sentinel lymph node (SLN) involvement in patients with tubular, cribriform, mucinous or papillary breast carcinoma and those related to non-SLN metastases if an SLN was positive. Multivariate analyses involved logistic and stepwise regressions. The SLNs harboured metastases in 85 of 572 cases, 78 of whom underwent axillary dissection; 19 presented non-SLN positive disease. Lack of lymphovascular invasion, a tumour size < or = 10 mm and a single SLN removed were the factors predicting an SLN metastasis rate <10%, and patients with these features could be candidates for no surgical axillary staging. A positive SLN proportion of < or = 50% and no lymphovascular invasion were associated with a <10% rate of non-SLN invasion; patients with a positive SLN and these features could be candidates for the omission of completion axillary dissection. The opposite presentation of these factors would mandate SLN biopsy and axillary dissection, respectively.  相似文献   
994.
AIM OF THE STUDY: The aim of this study was to calculate, for the first time, minimum provider volumes in total knee replacement using routine German data. MATERIALS AND METHODS: In patients with primary total knee replacement (TKR), the relationship between hospital volume per year and risk of "insufficient mobility" (primary quality indicator) and "wound infection" (secondary quality indicator) was calculated by means of logistic regression models. RESULTS: For both indicators, a statistically significant relationship between hospital volume and outcome could be demonstrated. Other risk factors such as age and ASA status also had a significant influence, but did not appear as important confounders. The risk for the secondary quality indicator "infection" decreased constantly with increasing hospital volume, thus the curve was very flat. This supports the hypothesis that high volume hospitals have a higher quality level than low volume hospitals. A threshold value could be calculated. However, the explanation value for hospital volume was too low to derive a threshold level that clearly discriminates between good and bad quality of care. The relationship between the primary quality indicator "insufficient mobility" and hospital volume unexpectedly showed a U-shaped distribution. This questions the concept of a minimum provider volume regulation for primary total knee replacement for the quality indicator "insufficient mobility". Therefore, in this case no quantitative threshold values were calculated. CONCLUSION: This analysis supports the hypothesis of a volume-outcome relationship in primary total knee replacement. However, a minimum provider volume that clearly discriminates between good and bad quality of care could not be calculated on the basis of these German quality assurance data.  相似文献   
995.
The survival rates of cementless femoral neck prostheses are lower than for cementless standard stems. Nevertheless, femoral neck prostheses have the advantage of preserving the femoral neck as well as not opening the diaphyseal marrow cavity. Should the need to change the endoprosthesis arise, a standard stem anchored in the diaphysis can be implanted after resection of the femoral neck. Since there is a relationship between surgical error and the learning curve for a new implant, the surgeon should be experienced in the field of total hip arthroplasty. In addition, the implantation of femoral neck prostheses is technically demanding. Therefore, careful patient selection is necessary and patients have to be informed of the advantages as well as the disadvantages of the different implant models.  相似文献   
996.
Giant cell tumor is a benign locally aggressive tumor with a high tendency to recurrence, with a small rate of pulmonary metastases. In 90% of cases the tumor occurs in the long bones, especially near the epiphysis. A case of a 37-year-old female with a recurrent giant cell tumor of the distal radius including the radioulnar articular surface, successfully treated with a wide resection and reconstruction of the articular surface between the radius, scaphoid, lunatum, and ulna by an iliac crest graft, is reported.  相似文献   
997.
Dermatology fulfills the prerequisites for telemedicine. An important application of telemedicine might be the field of dermatologic consultations. In this comparative study images of skin disease were taken of 120 patients hospitalized in the University Hospitals of Tuebingen to answer the following questions: (1) are the preconditions in daily routine given for teledermatology, (2) is there adequate agreement between the diagnoses reached in dermatologic consultations and with teledermatology, and (3) can the images be utilized for teaching purposes. Patient acceptance was very good and the images captured with a digital camera were easily obtained. The results of intraobserver analysis for the two teledermatologists without any knowledge of the patients' history were 70.2% and 46.4%, respectively, and with knowledge of the history 76.6% and 64.3%. The results of interobserver analysis without any knowledge of the patients' history were 46.4% and 57.2% and with knowledge of the history 64.3% and 66%, respectively. With the reduction of the image quality, reduced reliability of the diagnoses was observed. Seven of ten images could be used for teaching purposes. It was demonstrated that in dermatology telemedicine is applicable in many, but not in all patients who are referred for dermatologic consultations.  相似文献   
998.
Cutaneous adverse drug reactions (ADR) are common and encompass a broad clinical spectrum. Since the skin acts as a signaling organ for ADR, the dermatologist plays a key role in their diagnosis. Only a minor part of cutaneous ADR are due to underlying allergic mechanisms. Among these, delayed-type reactions such as maculopapular exanthems and immediate-type reactions such as urticaria and angioedema predominate. Risk factors for the development of cutaneous allergic ADR may be related to the patient (e.g. certain HLA-types), the drug (e.g. its reactivity), and underlying conditions (e.g. viral infections). Antibiotics, non-steroidal anti-inflammatory agents and anticonvulsive medications are most often reported to be causally related.  相似文献   
999.
The needs of children and families living with life-limiting and life-threatening illness are similar across all European countries. Meeting these needs requires a comprehensive and integrative approach, with the input of a skilled multidisciplinary paediatric team. It is essential that the core standards for paediatric palliative care recommended in this document of the European Association for Palliative Care (EAPC) now be implemented across Europe.  相似文献   
1000.
BACKGROUND: In a survey of German hospitals with obstetric units, data on anaesthesia and analgesia in obstetric pain management were collected. METHODS: At each of 918 hospitals with obstetric units, the head of the anaesthetic department received a questionnaire on obstetric pain management. RESULTS: The response rate was 47.1%. On average, there were 748+/-407 (median 663;1st/3rd quartiles 309/1,303) births per year and hospital, 69.4% with spontaneous vaginal delivery. Opioids were the systemic analgesics most frequently administered in the delivery rooms. Epidural analgesia (EA) was given to 17.5+/-12.6% of the parturients. The number of deliveries per annum had a significant influence on the frequency of EA (<500 deliveries/year: 12.7%, 500-1000/year: 18.6%, >1,000/year: 21.6%). The preferred local anaesthetics were ropivacaine und bupivacaine. When an opioid was given this was almost always sufentanil. In 16% of the responding hospitals adrenaline was added to the epidural test bolus. CONCLUSION: EA is the mainstream method of relieving labour pains in almost all German hospitals, but is used significantly more often in hospitals with rather high numbers of yearly deliveries than in hospitals in which there are few deliveries per year.  相似文献   
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