ABSTRACT
The present meta-analysis was conducted to determine the efficacy of hydroxyurea in patients with transfusion dependent major ß-thalassemia. The present meta-analysis was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and Meta-analyses of Observational Studies in Epidemiology (MOOSE) guidelines. A systematic search was carried out to evaluate the efficacy of hydroxyurea in patients with transfusion-dependent B-thalassaemia using electronic databases, including MEDLINE, Cochrane Central Register of Controlled Trials, and EMBASE. The keywords used to search for relevant studies included "hydroxyurea", "thalassemia", "transfusion-dependent", and "efficacy". Outcomes assessed in the present meta-analysis included transfusion in one year and intervals between transfusions (in days). Other outcomes assessed in the present meta-analysis were fetal hemoglobin (%), hemoglobin (%), and ferritin levels (ng/dl). Total of five studies were included in the analysis enrolling 294 patients with major B-thalassemia. The pooled analysis reported that the mean interval between transfusions was significantly higher in patients receiving hydroxyurea compared to those not receiving hydroxyurea (mean deviation {MD}: 10.07, 95% CI: 2.16, 17.99). Hemoglobin was significantly higher in patients receiving hydroxyurea compared to its counterparts (MD: 1.71, 95% CI: 0.84, 2.57). Patients receiving hydroxyurea had significantly lower ferritin levels compared to those not receiving hydroxyurea (MD: -299.65, 95% CI: -518.35, -80.96). These findings suggest that hydroxyurea may be a promising and cost-effective alternative to blood transfusions and iron chelation therapies for beta-thalassemia patients. However, the authors noted that further randomized controlled trials are needed to validate these findings and to determine the optimal dosages and treatment regimens for hydroxyurea in this patient population.
ABSTRACT
This meta-analysis aims to compare high-flow nasal cannula (HFNC) and conventional oxygen therapy (COT) post-extubation in pediatric cardiac surgical patients. The present meta-analysis was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Two authors independently searched three electronic databases including PubMed, Embase, and the Cochrane Library to identify relevant articles published in English from inception to February 2023. Searching was conducted using keywords and medical subject headings (MeSH), which included "conventional oxygen therapy," "high-flow nasal cannula," "extubation," "pediatrics," and "cardiac surgery." Our primary outcome was extubation failure defined as the need for reintubation within 24 to 72 hours after planned extubation. Secondary outcomes assessed in this meta-analysis included partial pressure of arterial oxygen (PaO2), partial pressure of arterial carbon dioxide (PaCO2), and the ratio of PaO2 and FiO2 (fraction of inspired oxygen). A total of three studies were included in the meta-analysis, with a total of 227 patients. No significant difference was found between the two groups (the HFNC group and the COT group) in terms of reintubation (RR: 0.88, 95% CI: 0.34, 2.30, p-value: 0.80). Pooled meta-analysis showed that PaO2 was significantly greater in patients receiving HFNC at six hours (MD: 33.73, 95% CI: 18.33, 49.14, p-value<0.001), at 12 hours (MD: 44.90, 95% CI: 28.59, 61.22, p-value<0.001) and at 24 hours (MD: 43.53, 95% CI: 29.16, 57.91, p-value<0.001) of extubation. PaCO2 was significantly lower in patients receiving HFNC at six hours (MD: -5.40, 95% CI: -7.94, -2.85, p-value<0.001) and at 12 hours (MD: -5.93, 95% CI: -9.78, -2.09, p-value<0.001) of extubation. However, no significant difference was reported between the two groups after 24 hours of extubation (MD: -0.84, 95% CI: -9.04, 7.37, p-value: 0.84) and PaO2/FiO2 was significantly greater in patients receiving HFNC at six hours (MD: 64.14, 95% CI: 36.10, 92.17, p-value<0.001), at 12 hours (MD: 70.73, 95% CI: 20.46, 121.01, p-value<0.001) and at 24 hours (MD: 82.18, 95% CI: 50.03, 114.32, p-value<0.001) of intubation. In conclusion, the meta-analysis revealed that compared with COT, HFNC significantly increased PaO2 and the ratio of PaO2 to FiO2, and decreased PaCO2. No significant differences were observed in the rate of reintubation between the two groups. This is the first meta-analysis comparing HFNC and COT in pediatric cardiac surgical patients.
ABSTRACT
Dubin-Johnson syndrome (DJS) is a rare autosomal recessive genetic disease caused by mutations in the bilirubin transporter MRP2. It is characterized by recurrent episodes of jaundice and conjugated hyperbilirubinemia. Numerous instances of hyperbilirubinemia disorders resembling Dubin-Johnson syndrome have been documented, but they differ in the clinical presentation, amount of conjugated bilirubin present, and their reaction to therapy. Most people with this syndrome do not have any symptoms, so their cases are often misdiagnosed and not properly taken care of. Here, we present a case of a teenage male patient who complained of recurring jaundice and abdominal pain. Further examination and testing revealed that the patient had been jaundiced since birth and had a family history of the condition. Conservative management was implemented, and follow-up demonstrated a positive prognosis. This case is a rare example of Dubin-Johnson syndrome, although patients with the condition generally have a normal life expectancy and only require conservative management.