Thrombopoietin receptor agonist switch in children with persistent and chronic severe immune thrombocytopenia: A retrospective analysis in a large tertiary center

We retrospectively analyzed sequential therapy with romiplostim and eltrombopag in 23 children with immune thrombocytopenia: switching from romiplostim to eltrombopag (10 patients) or vice versa (13 patients). The median age of patients at enrollment in the study was 5.6 years (2‐15 years). Switching from romiplostim to eltrombopag was effective in eight (80%) patients, whereas switching from eltrombopag to romiplostim was effective in eight (62%) patients. The response rate was similar in patients failing the first thrombopoietin receptor agonist and those who had previous response. To date, all responders continue to maintain platelets over 50 × 10⁹/L at 13‐39 months after switching.     

Clinical and biochemical spectrum of metabolic cardiomyopathy in Egyptian children

BackgroundInborn errors of metabolism (IEMs) commonly present with pediatric cardiomyopathy. Identification of the underlying cause is necessary as it may lead to improved outcomes.ObjectivesWe aimed to investigate the diagnostic rate, the clinical, and biochemical spectra of IEMs among Egyptian pediatric patients presenting with cardiomyopathy, and their outcome measures.MethodsWe retrospectively analyzed the clinical, biochemical, and radiological data of 1512 children diagnosed with cardiomyopathy at Cairo University Children''s Hospital over a 5-year duration.ResultsTwo hundred twenty-nine children were clinically suspected as IEMs and underwent metabolic workup. Nineteen different IEMs were confirmed in 57 (24.4%) of the suspected children. Their median age at presentation was 2.6 years and the majority had extra-cardiac manifestations. Hypertrophic cardiomyopathy represented 43/57 (75.4%) of confirmed cases, while dilated cardiomyopathy represented 13/57 (22.8%), and one patient presented with a mixed phenotype. Twenty- six patients (45.6%) survived, while 31 patients (54%) either died or were lost to follow up and assumed deceased.ConclusionsWe developed for the first time a database and a diagnostic scheme for metabolic cardiomyopathies in Egyptian children. With the recent introduction of enzyme replacement therapy, many metabolic disorders became treatable, thus establishing an early and accurate diagnosis is extremely important.     

The association between parents' stress and parental feeding practices and feeding styles: Systematic review and meta‐analysis of observational studies

In the extended UNICEF framework of early childhood nutrition, parents'' stress is associated with parental feeding style. However, no comprehensive review has examined the association between parents'' stress and feeding styles and practices. The objective of our review was to synthesise the current literature examining the association between parents'' stress and their feeding practices and/or styles, among parents of children ≤ 5 years old. We searched; MEDLINE, EMBASE, PSYCHINFO and CINAHL from 2019 to 2021. Two investigators independently extracted relevant data and assessed the study quality and the certainty of evidence. Data were pooled using generic inverse variance with fixed effects (<5 comparisons) or random effects (≥5 comparisons) and expressed as correlation coefficients with 95% confidence intervals (CI). Between study heterogeneity was assessed using Cochran''s Q and quantified with I ². We identified 6 longitudinal and 11 cross‐sectional studies, of which 4 studies provided sufficient data to be pooled. A very small correlation between general stress and restrictive feeding practices was observed (r = 0.06 [95% CI: 0.01−0.12]; no substantial heterogeneity (I ² = 0.00%, P _Q < 0.85, very low certainty). No correlation between general stress and feeding pressure was identified (r = 0.06 [95% CI: −0.02 to 0.15]). Results showed that both general and parenting stress were associated with suboptimal breastfeeding practices and unresponsive feeding styles. Conclusion: This study demonstrated a low‐to‐moderate quality of literature for the inclusion of parents'' stress in the extended UNICEF care model of child nutrition. Future research needs to explore this relationship longitudinally and in ethnic diverse populations to inform tailored interventions that promote responsive feeding practices.     

A Feasibility Study of High-Entropy Alloy Coating Deposition by Detonation Spraying Combined with Laser Melting

In this work, a new two-stage approach to the deposition of high-entropy alloy coatings is proposed. At the first stage, a composite precursor coating is formed by detonation spraying of the metal powder mixtures. At the second stage, the precursor coating is re-melted by a laser, and the formation of multi-component solid solution phases can be expected upon solidification. The feasibility of the proposed approach was validated using three different mixtures of Fe, Ni, Cu, Co and Al powders. It was shown that detonation spraying allows forming composite coatings with a uniform distribution of the lamellae of different metals. The results of the structural analysis of the laser-treated coatings suggest that complete alloying occurred in the melt and face-centered cubic solid solutions formed in the coatings upon cooling.     

肝癌组织中CLDN1的表达及其过表达对肝癌细胞恶性表型的影响

目的:探讨CLDN1在肝癌组织中的表达及其过表达对肝癌细胞恶性表型的影响.方法:通过免疫组化法及PCR法测定肝癌组织与癌旁组织中CLDN表达水平,建立CLDN1过表达细胞系,测定CLDN1过表达细胞株增殖能力、肿瘤细胞成球能力及干性相关标志物水平.结果:CLDN1在肝癌组织中表达阳性率为65.2％(58/89),在癌旁...     

Intrauterine Interventions for the Treatment of Twin Anemia-Polycythemia Sequence: A Systematic Review

BackgroundTwin anemia-polycythemia sequence (TAPS) is a complication of monochorionic, multiple gestation pregnancies in which blood shunting through placental anastomoses results in chronic anemia in one fetus and chronic polycythemia in another. The outcomes of different treatment modalities for TAPS are not well known.ObjectiveTo determine the outcomes of the intrauterine interventions used to treat TAPS.Study DesignA systematic literature search of MEDLINE, EMBASE, and CENTRAL was performed in June 2016. Primary outcomes were mortality, morbidity, and adverse perinatal outcomes. Data were summarized in the form of weighted means, and statistical difference was determined.ResultsTwenty-one articles were identified for inclusion in this review and were composed of 105 cases of TAPS. In the cases presented in the literature, there was no statistically significant difference in mortality, morbidity, or emergent Caesarean section rates between expectant management, intrauterine transfusion (IUT), and laser ablation therapy. Laser ablation therapy and IUT were found to have a significantly lower rate of adverse perinatal outcomes when compared to expectantly managed cases.ConclusionsThe literature looking into the treatment of TAPS is very limited, with no randomized controlled trials and only one includable comparative study. Based on the data in the case report and case study literature, there is no mortality difference between any of the treatment modalities. Expectant management may be associated with an increase in adverse perinatal outcomes when compared to laser therapy and IUT. More comparative studies are needed to assist clinicians in adopting an evidence-based approach to the treatment of TAPS.