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41.
It is well known the involvement of the gastrointestinal tract in Epidermolysis Bulhosa (EB) and its consequences on both nutrition and growth. We present a case of a young girl with EB diagnosed soon after birth, admitted to our emergency room with food impactation. The endoscopy revealed a double barrel esophagus. No medical or surgical therapy was started and after 2 months of enteric feeding, spontaneous resolution was observed at endoscopy. Skin biopsy was compatible with EB Simplex. According to the literature EB Simplex is the subtype the least associated with GI tract involvement, presenting with dysphagia in only 2% of the patients, and with the lowest cumulative risk of esophageal stricturing.  相似文献   
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Type II deiodinase (D2) activates thyroid hormone by converting thyroxine (T4) to 3,5,3′-triiodothyronine (T3). This allows plasma T4 to signal a negative feedback loop that inhibits production of thyrotropin-releasing hormone (TRH) in the mediobasal hypothalamus (MBH) and thyroid-stimulating hormone (TSH) in the pituitary. To determine the relative contributions of these D2 pathways in the feedback loop, we developed 2 mouse strains with pituitary- and astrocyte-specific D2 knockdown (pit-D2 KO and astro-D2 KO mice, respectively). The pit-D2 KO mice had normal serum T3 and were systemically euthyroid, but exhibited an approximately 3-fold elevation in serum TSH levels and a 40% reduction in biological activity. This was the result of elevated serum T4 that increased D2-mediated T3 production in the MBH, thus decreasing Trh mRNA. That tanycytes, not astrocytes, are the cells within the MBH that mediate T4-to-T3 conversion was defined by studies using the astro-D2 KO mice. Despite near-complete loss of brain D2, tanycyte D2 was preserved in astro-D2 KO mice at levels that were sufficient to maintain both the T4-dependent negative feedback loop and thyroid economy. Taken together, these data demonstrated that the hypothalamic-thyroid axis is wired to maintain normal plasma T3 levels, which is achieved through coordination of T4-to-T3 conversion between thyrotrophs and tanycytes.  相似文献   
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Aims and objectives. To examine the prevalence of aggression against healthcare professionals and to determine the possible impact that violent episodes have on healthcare professionals in terms of loss of enthusiasm and involvement towards work. The objective was to analyse the percentage of occupational assault against professionals’ aggression in different types of healthcare services, differentiating between physical and verbal aggression as a possible variable in detecting burnout in doctors and nursing professionals. Background. Leiter and Maslach have explored a double process model of burnout not only based on exhaustion by overload, but also based on personal and organisational value conflicts (community, rewards or values). Moreover, Whittington has obtained conclusive results about the possible relationship between violence and burnout in mental health nurses. Design. A retrospective study was performed in three hospitals and 22 primary care centres in Spain (n = 1·826). Methods. Through different questionnaires, we have explored the relationship between aggression suffered by healthcare workers and burnout. Results. Eleven percent of respondents had been physically assaulted on at least one occasion, whilst 34·4% had suffered threats and intimidation on at least one occasion and 36·6% had been subjected to insults. Both forms of violence, physical and non‐physical aggression, showed significant correlations with symptoms of burnout (emotional exhaustion, depersonalisation and inefficacy). Conclusions. The survey showed evidence of a double process: (1) by which excess workload helps predict burnout, and (2) by which a mismatch in the congruence of values, or interpersonal conflict, contributes in a meaningful way to each of the dimensions of burnout, adding overhead to the process of exhaustion–cynicism–lack of realisation. Relevance to clinical practice. Studies indicate that health professionals are some of the most exposed to disorders steaming from psychosocial risks and a high comorbidity: anxiety, depression, etc. There is a clear need for accurate instruments of evaluation to detect not only the burnout but also the areas that cause it. Professional exhaustion caused by aggression or other factors can reflect a deterioration in the healthcare relationship.  相似文献   
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Background Preliminary data have shown encouraging results of a single intratumoral radiopharmaceutical injection that enables both sentinel node biopsy and probe-guided excision of the primary tumor in patients with nonpalpable breast cancer. The aim of the study was to evaluate this approach in a large group of patients. Methods Lymphoscintigraphy was performed in 368 patients with nonpalpable breast cancer after intratumoral injection of 99mTc-nanocolloid (.2 mL, 123 MBq, 3.3 mCi) guided by ultrasound or stereotaxis. The sentinel node was pursued with the aid of vital blue dye (1.0 mL, intratumoral) and a gamma ray detection probe. In case of breast-conserving surgery, the probe was used to guide the excision. Results At least one sentinel node could be identified intraoperatively in 357 patients (97%), of whom 69 had involved nodes (19%). Age over 60 years was associated with less frequent nonaxillary lymphatic drainage and absence of internal mammary chain dissemination. Tumor-free margins were obtained in 262 (89%) of the 293 patients who underwent segmental excision. Re-excision of the primary tumor bed was performed in six patients (2%). During a median follow-up of 22 months, one breast recurrence and one axillary recurrence were observed. Conclusions Lymphatic mapping and probe-guided tumor excision of nonpalpable breast cancer by intralesional administration of a single dose of 99mTc-nanocolloid and blue dye resulted in 97% identification of the sentinel node and in tumor-free margins in 89% of the patients who underwent breast-conserving surgery. Longer follow-up is needed to substantiate the accuracy and safety of this technique.  相似文献   
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Patients with chronic renal failure who are undergoing dialysis and kidney transplant recipients are susceptible to infection for several reasons. In this study, the profile of infections in patients with chronic renal failure and kidney transplant recipients treated at a hospital in northern Paraná, Brazil, from 2007 to 2009 was examined. The study involved 187 patients: 59 kidney transplant recipients and 128 patients undergoing dialysis. The frequency of infection was 25% (32/128) in dialysis patients and 8% (5/59) in transplant recipients (P = .008). Staphylococcus aureus was the most prevalent infectious agent, cultured from 27% (13/48) of samples, followed by Escherichia coli at 17% (8/48). All isolates of S aureus were sensitive to vancomycin and resistant to penicillin, and 43% were resistant to oxacillin. Most S aureus samples (43%) were isolated from cultures of blood samples. As for the E coli, 75% were resistant to cephalothin and 38% were resistant to sulfamethoxazole/trimethoprim. Most isolates of E coli (62%) were cultured from specimens of patients with suspected urinary tract infection.  相似文献   
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Negative pressure wound therapy (NPWT) is becoming routine for the preparation of wounds prior to grafting for wound closure. We have been using both foam- and gauze-based NPWT to prepare wounds for closure prior to skin grafting and have obtained similar proportions of closed wounds; 7/7 for wounds treated with gauze-based NPWT and 11/11 for wounds treated with foam-based NPWT. In our follow-up consultations we observed that skin grafts on the foam-treated patients were less pliable than those on the gauze-treated patients. To assess what the mechanism of this effect might be, we compared the specific details of the treatments of both 11 foam and 7 gauze patients, including depth, location, patients' age and co-morbidity; biopsies of granulation and scar tissue were taken and stained with haematoxylin-eosin and by Masson's trichrome staining and conducted ultrasound analysis of the closed wounds, to see if there were features which explained those effects. All foam patients were treated at -125 mm Hg for an average of 25·9 days before skin grafts were applied. All gauze patients were treated at -80 mm Hg for an average of 24·7 days before skin grafts were applied. Biopsies of granulation tissue prior to skin grafting from five foam and four gauze-based NPWT patients did not reveal any obvious histological differences between the treatments. Ultrasound analysis of the skin-grafted wounds showed an average depth of scar tissue of 18 mm in the wound beds of the foam-treated wounds and 7 mm in the gauze-treated ones. Biopsies taken on the scar tissue after treatment with the gauze showed a minor tissue thickness and disorganisation and less sclerotic components. The findings of this preliminary analysis suggest that foam-based NPWT may induce a thicker layer of scar tissue beneath skin grafts than gauze-based NPWT which might explain a reduced pliability of the reconstructed bed. At present it is unclear which mechanism might be responsible for the difference in pressure (-125 versus -80 mm Hg), either the length of the time taken to reconstruct the wound bed or the intrinsic nature of the foam or gauze on the tissue surface. Prospective studies are necessary to investigate whether these preliminary observations are confirmed and to investigate what the mechanism might be.  相似文献   
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