RESUMEN
Carney complex is rare neoplastic disorder. Intracardiac myxoma presenting the most common non-cutaneous lesions in this complex. We are reporting a 31-year-old Saudi female known case of Carney complex presented with asymptomatic biatrial myxoma that was identified on routine transthoracic echocardiogram, and was successfully excised. However, these patients need a careful surveillance in order to detect any new masses and prevent their complications.
RESUMEN
ß-thalassemia is characterized by the faulty generation of hemoglobin resulting in an elevated α/ß globin ratio; this led to several patients needing red blood cell (RBC) transfusions for the rest of their lives. Luspatercept is an erythroid maturation test for treating various types of anemia, including ß-thalassemia. It inhibits the Smad2/3 cascade and treats ß-thalassemia by downregulating the transforming growth factor-beta (TGF-ß) pathway. Luspatercept was evaluated in randomized controlled trials (RCTs). However, there is still limited data. Therefore, the study aims to review the current literature to assess the efficacy of luspatercept in cure ß-thalassemia and its safety. From 2015 to 2022, searches were undertaken in PubMed, Google Scholar, and Cochrane. Only RCTs published in English were eligible for inclusion. The Cochrane Collaboration tool for bias assessment was used to analyze the quality of the publications. Our search strategy revealed 94 publications, of which 12 full-text papers were read and five were chosen for this review.All five trials included 1161 participants. Of whom, 153 (13.18%) entered phase 2, and 1008 (86.82%) entered phase 3. Two articles included 153 participants, of whom 70 (45.75%) were transfusion-dependent beta-thalassemia (TD) and 83 (54.25%) were non-transfusion-dependent beta-thalassemia (NTD) of phase 2. Three articles included 1008 participants, of whom 672 (66.67%) were given luspatercept and 336 (33.33%) were given a placebo. All participants in RCTs were 18 years of age or older. In phase 2, 0.2 to 1.25 mg/kg of luspatercept was given, and in phase 3, 1.0 to 1.25 mg/kg of luspatercept was given once every three weeks. In beta-thalassemia patients, luspatercept was more effective than a placebo and well tolerated. The high dose has shown promising results in the erythroid response, measured by a drop in blood transfusions or an average rise in hemoglobin levels. Luspatercept might make patients less likely to need RBC transfusions, improve their clinical results, and improve their quality of life. Adverse events were hyperuricemia, arthralgia, dizziness, influenza hypertension, and bone pain, but they were manageable.
RESUMEN
Background and objectives: Following cardiac surgery, acute kidney injury (AKI) is a well-known complication that increases morbidity and mortality. This study was carried out to determine the factors associated with acute kidney injury and to assess the predictive value of three predictive scores for the development of AKI post-cardiac surgery in the Saudi community. Methods: In this retrospective study, the medical records of patients aged 18 years and above who underwent cardiac surgery on cardiopulmonary bypass (CPB) at Saud Albabtin Cardiac Center between January 2018 and March 2021 were reviewed. The first stage of both Kidney Disease Improving Global Outcomes (KDIGO) criteria and the risk, injury, failure, loss, end-stage (RIFLE) criteria were used to define AKI. The predicting value for acute kidney injury following cardiac surgery (AKICS score) and Renal replacement therapy for acute kidney injury (RRT-AKI) (Cleveland score, and SRI) were evaluated by area under receiver operating characteristic curve (AUROC) for the discrimination and Hosmer-Lemeshow test for the calibration. Results: Among the 329 patients evaluated, the total postoperative incidence of acute kidney injury was 26.4%. Moreover, the incidence of RRT-AKI was 2.1%. Using multivariate logistic analysis, the factors independently associated with AKI were CABG on pump-beating heart, presence of chronic kidney disease, pre-operative anemia, prolonged bypass time, and post-operative exposure to inotropes or vasopressors. For the prediction of CSA-AKI, the discrimination of AKICS (AUROC = 0.689) was poor, while the calibration (x2 = 9.380, P = 0.311) was fair. For RRT-AKI prediction, the discrimination of Cleveland score (AUROC = 0.717) was fair while the discrimination of SRI (AUROC = 0. 681) was poor. On the other hand, the calibration for both of them was fair (Cleveland score x2 = 3.339, P = 0.342; SRI x2 = 7.326, P = 0.120). Conclusion: In this single-center study, SRI score demonstrated a reasonably good prediction of RRT-AKI incidence. However, further researches are required to investigate the perioperative factors in order to create a unique risk score model that may be used in a population with widespread comorbidities.