Activiteit

We describe how infants and children with hereditary and acquired autoimmune thrombotic thrombocytopenic purpura (TTP) initially present and how they can be promptly diagnosed and effectively managed. These are uncommon disorders that are commonly misdiagnosed and can be rapidly fatal. TTP is caused by a severe deficiency of the plasma protease, A disintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS13). Measurement of ADAMTS13 activity is becoming easily accessible. A common presentation of hereditary TTP is neonatal severe hemolysis and hyperbilirubinemia. However, the median age of diagnosis is not until 5.5 years. Plasma is effective treatment for exacerbations and for prophylaxis. Plasma may be replaced by recombinant ADAMTS13 when it becomes available. Acquired TTP is more frequent in older children, in whom it is more common in girls and is commonly associated with systemic lupus erythematosus. For acquired TTP, plasma exchange and immunosuppression are the current treatment for acute episodes; caplacizumab is now commonly used in adults and may replace plasma exchange.The Society for Craniofacial Genetics and Developmental Biology (SCGDB) held its 43rd annual meeting in a virtual format on October 19-20, 2020. The SCGDB meeting included the presentation of the SCGDB Distinguished Scientists in Craniofacial Research Awards to Marilyn Jones and Kerstin Ludwig and four scientific sessions on the molecular regulation of craniofacial development, craniofacial morphogenesis, translational craniofacial biology, and signaling during craniofacial development. The meeting also included workshops on career development, NIH/NIDCR funding, and the utility of the FaceBase database, as well as two poster sessions. Over 190 attendees from 21 states, representing over 50 different scientific institutions, participated. This diverse group of scientists included cell biologists, developmental biologists, and clinical geneticists. While in-person interactions were missed due to the virtual meeting format imposed by the COVID-19 pandemic, the meeting platform provided ample opportunities for participant interactions and discussions, thus strengthening the community.

To characterise medical, obstetric and demographic risk factors associated with nulliparous, term, singleton, vertex (NTSV) caesarean birth.

Cross-sectional study.

United States delivery hospitalisations.

NTSV births in 2016-18 US natality data.

This study analysed a national sample of natality data generated by the United States National Vital Statistics System. NTSV deliveries were identified. The primary outcome was caesarean birth. Risk factors including maternal age, body mass index (BMI) and pregestational diabetes were analysed. Multivariable log-linear regression models analysed factors associated with NTSV caesarean with adjusted risk ratios (aRR) as measures of effect.

Of 11 622 400 deliveries, 3 764 707 met NTSV criteria, and their caesarean section rate was 25.9%. Maternal age 35-39 years (aRR 1.51, 95% CI 1.50-1.52) and 40-54 years (aRR 2.03, 95% 2.00-2.05) compared with age 19-34 years; BMI 25 to <30 kg/m2 (aRR 1.32, 95% CI 1.31-1.33), 30 to <35 kg/m2 (aRR 1.57 95% CI 1.56-1.58), 35 to <40 kg/m2 (aRR 1.82, 95% CI 1.80-1.83) and ≥40 kg/m2 (aRR 2.17, 95% CI 2.15-2.19) compared with BMI 18.5-24.9 kg/m2; and pregestational diabetes (aRR 1.54, 95% CI 1.51-1.57) were all associated with increased risk. Risk factors allowed stratification of patients into high-risk versus low-risk groups. The NTSV caesarean rate was 37.9% in women who had one or more of the following characteristics age ≥35 years, BMI ≥30 kg/m2 or pregestational diabetes. In comparison, the NTSV caesarean rate was 20.8% among women without any of these three risk factors (P < 0.01).

Among NTSV births, BMI, maternal age and medical conditions are important risk factors for caesarean delivery.

Among NTSV births, BMI, maternal age and medical conditions are important risk factors for caesarean delivery.A new extraction method of polyphenols from honey using a biodegradable resin was developed and compared with the common commercial resin amberlite XAD2. For this purpose, three honey samples of Algerian origin were selected for the different physicochemical and biochemical parameters study. see more After extraction of the target compounds by both resins, the polyphenol content was determined, the antioxidant activity was tested, and liquid chromatography-mass spectrometry analyses were performed for identification and quantification. The results showed that physicochemical and biochemical parameters meet the norms of the International Honey Commission, and the H1 sample seemed to be of high quality. The optimal conditions of extraction by biodegradable resin were a pH of 3, an adsorption dose of 40 g/L, a contact time of 50 min, an extraction temperature of 60°C, and no stirring. The regeneration and reuse number of both resins was three cycles. The polyphenol contents demonstrated a higher extraction efficiency of biosorbent than of XAD2, especially in H1. Liquid chromatography-mass spectrometry analyses allowed for the identification and quantification of 15 compounds in the different honey samples extracted using both resins and the most abundant compound was 3,4,5-trimethoxybenzoic acid. In addition, the biosorbent extracts showed stronger antioxidant activities than the XAD2 extracts.Direct allylic C-H thiolation is straightforward for allylic C(sp3 )-S bond formation. However, strong interactions between thiol and transition metal catalysts lead to deactivation of the catalytic cycle or oxidation of sulfur atom under oxidative condition. Thus, direct allylic C(sp3 )-H thiolation has proved difficult. Represented herein is an exceptional for direct, efficient, atom- and step-economic thiolation of allylic C(sp3 )-H and thiol S-H under visible light irradiation. Radical trapping experiments and electron paramagnetic resonance (EPR) spectroscopy identified the allylic radical and thiyl radical generated on the surface of photocatalyst quantum dots (QDs). The C-S bond formation does not require external oxidants and radical initiators, and hydrogen (H2 ) is produced as byproduct. When vinylic C(sp2 )-H was used instead of allylic C(sp3 )-H bond, the radical-radical cross-coupling of C(sp2 )-H and S-H was achieved with liberation of H2 . Such a unique transformation opens up a door toward direct C-H and S-H coupling for valuable organosulfur chemistry.