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Clinical Rheumatology - Coronavirus disease-19 (COVID-19) is a global pandemic that is caused by COVID-19 virus, which was initially identified in December 2019 in Wuhan, China. Vaccination is one...  相似文献   
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Familial hypomagnesemia with hypercalciuria and nephrocalcinosis   总被引:2,自引:0,他引:2  
Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is a rare autosomal recessive tubular disorder that is frequently associated with progressive renal failure. The primary defect is related to impaired tubular reabsorption of magnesium (Mg) and calcium (Ca) in the thick ascending limb of Henle's loop. We have studied seven Arab patients with this syndrome who belong to four different families. The mean age at first presentation was 1.5+/-1.3 years (range 0.1-3 years) and at diagnosis 5.9+/-4.3 years (range 0.5-12 years). The presenting features were convulsions and carpopedal spasms (5 patients), polydipsia and polyuria (2 patients), rickets (2 patients), and recurrent urinary tract infections (1 patient). Bilateral nephrocalcinosis was observed in all patients. All patients had hypomagnesemia with a mean serum Mg of 0.45+/-0.09 mmol/l, an inappropriately high urine Mg of 2.07+/-0.73 mmol/24 h or fractional excretion of 15.3+/-7.1%, high urine Ca excretion of 4.1+/-1.2 mmol/24 h or urine Ca to creatinine ratio of 2.6+/-1.6, and normal serum potassium level of 4.4+/-0.34 mmol/l. All patients received Mg supplements and thiazide but exhibited slow worsening of their kidney function. After a mean follow-up of 4.4+/-3.9 years, one patient progressed to end-stage renal failure (ESRF). In conclusion, we report seven Arab patients with FHHNC syndrome. The clinical and biochemical data were similar to previous reports. However, they tend to show a slower rate of progression to ESRF.  相似文献   
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IntroductionAppropriate prescribing of thromboprophylaxis according to guidelines’ recommendations can heighten over- or underutilization risk. The study intended to evaluate the safety and effectiveness of appropriate/inappropriate thromboprophylaxis use among hospitalized elderly medical patients.MethodsA retrospective observational cohort study was conducted, including patients who were ≥60 years old, hospitalized for an acute medical illness that required hospitalization in a medical ward for >48 h, and received thromboprophylaxis. Against the American College of Chest Physicians guidelines, the thromboprophylaxis use appropriateness was assessed.ResultsA total of 370 patients met the inclusion criteria, in 71.9% of whom thromboprophylaxis use was appropriate. The mean age of the included patients was 75 years (±9.1), and 72.4% of them were at high risk of venous thromboembolism (VTE), and almost all these patients received appropriate thromboprophylaxis. The occurrence of bleeding was significantly higher in the appropriate use group during hospitalization than the inappropriate use group (11.7% vs. 2.9%, p = 0.009); the majority of these bleeding events were classified as major. There were no differences in VTE events during hospitalization or 90 days all-cause mortality between the two groups.ConclusionThe study demonstrates high prescribers’ compliance with recommendations in high-risk patients. In patients at low risk for VTE, the overutilization of thromboprophylaxis did not increase their bleeding risk. This study suggests that the benefits of thromboprophylaxis in elderly patients, regardless of their VTE risk, may outweigh the risk of bleeding.  相似文献   
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Hand hygiene is one of the effective measures for reducing the transmission of infections. Alcohol-based hand sanitizers containing ethanol or isopropanol are considered efficient alternatives to handwashing with water and soap. Despite being effective against a broad-spectrum of microbes, fining an effective alternative to the alcohol-based hand sanitizers became a necessity owning to the limitations associated with their use, such as skin dryness, irritant contact dermatitis, and intoxication upon their accidental ingestion. Furthermore, in certain circumstances when the demand for alcohol exceeds the supply, like in the current COVID19 pandemic, formulating an effective non-alcoholic hand sanitizer would be a potential solution. Therefore, in this study, a non-alcoholic hand sanitizer containing benzalkonium chloride (BKC) as an active ingredient was prepared and evaluated as a less irritant and more persistent hand sanitizer gel. The hand gel was characterized by pH, viscosity, and spreadability. Results showed that this product has low viscosity, high spreadability and pH of 6.3, which is less likely to cause skin irritation. The antibacterial assessment (zone of inhibition) of the BKC-based hand sanitizer demonstrated antibacterial activities against nine out of eleven gram-positive and gram-negative bacterial strains, while the acceptability study on ten participants showed no signs of skin irritation nor redness upon its application. Consequently, this non-alcoholic based hand sanitizer is suggested as a potential alternative to alcohol-based hand gels.  相似文献   
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Rituximab has been used over the last decade as a rescue therapy for refractory cases of nephrotic syndrome (NS). Here we report the use of rituximab in four children with idiopathic steroid-resistant nephrotic syndrome (SRNS) with various histological backgrounds (two cases with focal segmental glomerulosclerosis, one case with IgM nephropathy, and one case with minimal change disease), who failed to respond to other immunsuppressions. Their median age (range) was 10 (8–11) years. NPHS2 genetic mutation was negative in all of them. All patients received a single dose of rituximab (375 mg/m2) and achieved complete B cell depletion as CD19 was <1% for 3 months following rituximab infusion. Only one patient achieved non-sustained remission as he relapsed after 4 months despite zero CD19 level. Patients received no further doses of rituximab as B cell was depleted in the peripheral circulation. We conclude that a single dose of rituximab was not effective in inducing sustained remission in children with idiopathic SRNS, despite complete B cell depletion in the peripheral circulation. Further doses might be indicated to deplete non-circulating B cells.  相似文献   
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Background/aimCardiac surgery is considered one of the conditions that require a transfusion of blood and blood products in large amount. Infections are one of the most common complications after cardiac surgery. The aim of this study is to assess the impact of blood transfusion on major infections after isolated coronary artery bypass surgery (CABG).MethodsA retrospective cohort study was conducted at King Abdulaziz Cardiac Center. Eligible adult patients, aged >18 years, who underwent an isolated CABG from 2015 to 2016, were included. Patient demographic information, as well as pre-, intra-, and postoperative data were collected from the electronic hospital information system charts and perfusion records. For data analysis, categorical pre- and postoperative variables were summarized by frequencies and percentages, whereas for continuous variables, means and standard deviation or median and interquartile ranges were used.ResultsThe sample size was 459 patients. Red blood cells (RBCs) were transfused in 60.1% of the patients, and the median number of units transfused per patient was 2. The mean hemoglobin threshold for transfusion was 8.2 (standard deviation ± 3.6) g/dL. The mean EuroSCORE of RBC recipients was 3.8 ± 5.9% and that of non-RBC recipients was 2.0 ± 2.0%. In both groups (RBC recipients and non-RBC recipients), the most frequent infections after CABG were pneumonia (12% and 8.7%, respectively), deep surgical site infection (3.6% and 0.5%, respectively), and superficial sternal infection (6.9% and 3.8%, respectively), with a statistically significant difference (all p < 0.05). Patients receiving a blood transfusion at any stage during the intraoperative or postoperative period were 2.6 times more likely to develop an infection compared with those who did not receive a blood transfusion. The recipients of a blood transfusion experienced a longer hospital stay compared with the non-recipients at 11.5 ± 9.8 days versus 8.7 ± 3.4 days, respectively.ConclusionsBlood transfusion appears to increase the risk of infection post-CABG. However, increased understanding of the role of other potential clinical confounding variables that may impact the infection rate is required. We recommend management strategies that limit RBC transfusion.  相似文献   
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