Glucose-6-phosphate dehydrogenase deficiency is a common monogenic inheritance disorder, with extensive distribution around the world and very high gene frequency. Acute hemolysis may lead to rapidly rising of serum total bilirubin concentrations and may eventually result in bilirubin encephalopathy.The hyperbilirubinemia is the result of abnormal bilirubin metabolism due to complex interactions between G-6-phosphate dehydrogenase deficiency and the genetic co-expression. Neonatal unconjugated hyperbilirubinemia is one of the most common conditions encountered by the practicing pediatricians.

Objectives To summarize screening and therapeutic effects of congenital hypothyroidism (CH) in Zhongshan. Methods The thyroid stimulating hormone (TSH) concentration in dried heel blood samples on iflter paper was detected using time-resolved fluorescence immunoassay. The cases of positive screening tests were called back for further examination of venous blood TSH concentration using chemiluminescence method. Fifty-four children with permanent CH treated routinely for 2 years (CH group) and 120 age-gender matched health children (control group) were recruited. The physical development (height, body weight) was monitored. The neurodevelopment and temperament type were tested using Pediatric Nneuropsychological Development Assessment and Children's Temperament Scale respectively at 6 and 24 months after birth. Results Two hundred eight-five thousand two hundred forty-two neonates were screened. One hundred and forty cases were confirmed and the incidence rate was 1/2037. There was no statistical difference in length-for-age z score (LAZ) and weight-for-age z score (WAZ) between CH and control group (P>0.05). The neurodevelopment in CH group was normal, but gross motor development was worse than that in control group (P<0.05). The temperament type and distribution had statistical difference between CH and control group (P<0.05). The percentage of the dififcult type and central dififcult type was increased in CH group as compared to control group, especially in the activity, adaptability, reaction intensity and perseverance (P<0.05). Conclusions The physical and neurodevelopment are nearly normal in patients with CH after early supplementation, but the psychological behavior problems need to be focused on in the process of intervention.

Enzyme-driven micro/nanomotors consuming in situ chemical fuels have attracted lots of attention for biomedical applications. However, motor systems composed by organism-derived organics that maximize the therapeutic efficacy of enzymatic products remain challenging. Herein, swimming proteomotors based on biocompatible urease and human serum albumin are constructed for enhanced antitumor therapy via active motion and ammonia amplification. By decomposing urea into carbon dioxide and ammonia, the designed proteomotors are endowed with self-propulsive capability, which leads to improved internalization and enhanced penetration in vitro. As a glutamine synthetase inhibitor, the loaded l-methionine sulfoximine further prevents the conversion of toxic ammonia into non-toxic glutamine in both tumor and stromal cells, resulting in local ammonia amplification. After intravesical instillation, the proteomotors achieve longer bladder retention and thus significantly inhibit the growth of orthotopic bladder tumor in vivo without adverse effects. We envision that the as-developed swimming proteomotors with amplification of the product toxicity may be a potential platform for active cancer treatment.