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51.
The main clinical features of tyrosinemia type 1 usually appear in the first months of life, including fever, diarrhea, vomiting, liver involvement, growth failure, and renal proximal tubulopathy with subsequent hypophosphatemic rickets. An early diagnosis is crucial in order to provide specific management and to prevent complications. Here, we report on two cases referred primarily to pediatric nephrologists for the diagnosis of “neonatal tubulopathy” and management of “X-linked hypophosphatemia (XLH),” respectively. Our aim is to emphasize that (1) even a mixed tubulopathy can reveal tyrosinemia, and (2) tyrosinemia is a classic differential diagnosis of XLH that should not be forgotten, especially in the era of the anti-FGF23 burosumab.  相似文献   
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Clinical Rheumatology - Since December 2019, the pandemic caused by coronavirus disease 2019 (COVID-19) raises a real public health problem. COVID-19 appeared in Wuhan (Hubei province) in China....  相似文献   
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Objective

To discover the experiences of end-of-life patients attended by the emergency services, through the discourse of the family caregivers who accompanied the family member in this care transit.

Method

A qualitative approach study, based on the paradigm of hermeneutical phenomenology. In total, 81 family caregivers participated. The techniques used were the in-depth interview and the discussion group, with a total of 5 discussion groups and 41 interviews. The period of data collection was carried out between January 2013 and June 2014.

Results

In the network of discourses obtained with respect to “Urgent Care”, all the codes were grouped in relation to a single argumentative line: deficiencies in urgent care. Among them, we found different dimensions that are established depending on the different times of care, or the different determinant aspects of these deficiencies: disorganization of the care received, lack of experience of the professionals in emergencies, application of general protocols in the emergency services, inadequate care in the treatment received, delays in emergency care.

Conclusions

In general, we highlight the dissatisfaction of the family members with respect to the care received from the emergency services. The needs of these types of situation are not covered from these services and are of low quality. Therefore, it is necessary to reorient the care protocols for these patients.  相似文献   
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Introduction

The fast track / ultra-fast-track protocols are techniques used to optimise the patient care process and a quick recovery after cardiac surgery. They are one of the mainstays of efficient practice. With their use, the length of hospital and intensive care unit (ICU) stays are reduced, with a direct impact on costs and the quality of the health service.

Objective

To compare the length of stay in the ICU, length of hospital stay, and post-operative mortality in ultra-fast-track extubated (uFTE) patients and those with conventional extubation (CE) after cardiac surgery.

Methods

Longitudinal, analytical, retrospective study was conducted, with the period between the time of surgery and discharge being included as the study period.

Results

A total of 396 patients older than 18 years who required cardiac surgery were included, of whom 207 patients had (uFTE) and 189 had CE. Although the groups were not comparable due to the statistical differences found, when performing the multivariate adjustment, uFTE maintained its statistical independence and was associated with lower cardiovascular morbidity, such as myocardial ischaemia (95% CI: 0.37-0.86; P = .01) and lower post-surgical vasopressor requirement (95% CI: 0.18-0.49; P < .01). No significant differences were found in the length of hospital stay, ICU stay, or post-operative mortality in the ICU.

Conclusion

Implementing the uFTE strategy, decreases cardiovascular morbidity and vasopressor requirement. The change to uFTE should be accompanied by changes in models and practices in patient recovery to standardised protocols. This study shows that uFTE did not reduce the length of ICU stay, hospital stay, or mortality.  相似文献   
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