Comparative analysis of cystic biliary atresia and choledochal cysts.

Objective: Cystic biliary atresia (CBA) is a rare and peculiar type of biliary atresia (BA) that is easily confused with infantile choledochal cysts (CCs). This study explored information for early CBA diagnosis and treatment. Method: The authors retrospectively analyzed the clinical data of 32 children with hilar cysts from January 2013 to May 2021. According to the diagnosis, they were divided into the CBA (n = 12) and CC (n = 20) groups. Patient features, biochemical indexes, preoperative ultrasound characteristics, cholangiography features, and intraoperative findings were analyzed and compared between the two groups. Results: The alanine aminotransferase, aspartate aminotransferase, total bilirubin, and direct bilirubin levels in the CBA group were higher than in the CCs group (P < 0.05). Additionally, B-mode ultrasound showed a cystic mass in front of the hepatic hilum, and the cyst size was much smaller in the CBA group compared with the CC group (2.2 ± 1.3 cm vs. 6.0 ± 2.2 cm, P < 0.001). Among all of the parameters, cyst width was the most accurate for identifying CBA and CCs. A cutoff value of 2.5 cm (area under the curve, 0.98, P < 0.001) showed 90.9% sensitivity and 95% specificity for cyst size. Conclusion: For children with early-onset severe jaundice, and if the width of the cystic mass was ≤2.5 cm, a diagnosis of CBA was highly likely. Early cholangiography and surgical treatment are necessary for the effective treatment of these infants.

The abnormal phosphorylation of the Rac1, Lim-kinase 1, and Cofilin proteins in the pathogenesis of Hirschsprung's disease.

Rac1 can affect the migration of neural crest cells by regulating the polymerization of actin and the membrane formation process. But the role of the Rac1 signaling pathway in the pathogenesis of Hirschsprung's disease (HSCR) remains unclear. In order to investigate the mechanism of the abnormal protein phosphorylation of Rac1, Lim-kinase 1 (Limk1) and Cofilin involved in the pathogenesis of HSCR. The protein phosphorylation levels of these proteins were detected by Western blot in 30 samples of HSCR narrow segment, 30 samples of transitional segment tissues, and 14 samples of normal intestinal tissues. Subsequently, in the SH-SY5Y human neuroblastoma cell line, a Rac1, Limk1, and Cofilin inhibitor group, a Rac1 overexpression group (PDGF-BB group), a Rac1 overexpression group + a Limk1 inhibitor group (P-B group), a Rac1 overexpression group + a Cofilin inhibitor group (P-C group) were established. The results showed that the expressions of p-Rac1, p-Limk1, and p-Cofilin in HSCR narrow segment and transitional segment were lower than those in normal intestine (p < 0.05). The expression levels of p-Rac1, p-Limk1, and p-Cofilin in the relative inhibitor group were significantly lower than those in the control group (p < 0.05), and the proliferation and migration levels in the control group and Rac1 overexpression group were significantly higher than those in the Rac1, Limk1, and Cofilin inhibitor group (p < 0.05). In conclusion, the decreased phosphorylation of the Rac1/Limk1/Cofilin signaling pathway in HSCR could inhibit the proliferation and migration of SH-SY5Y cells, and this might be associated with the pathogenesis of HSCR.

Expression of the P2Y2 receptor in the terminal rectum of fetal rats with anorectal malformation.

OBJECTIVE: The expression and distribution of a subtype of purine receptors (P2Y2) in the terminal rectum of fetal rats with anorectal malformations (ARM) were examined to investigate their possible impact on the development of the enteric nervous system (ENS). METHODS: Pregnant Sprague-Dawley rats were randomly divided into a control group (5 rats) and an experimental group (20 rats). The experimental group was treated with ethylene thiourea (ETU). On gestational day 20, the intrauterine fetal rats were collected from both groups of pregnant rats. Sagittal sections of the pelvic perinea were stained with HE. P2Y2 protein and mRNA expression in the terminal recta of the fetal rats in the control group, the ARM group, and the ETU-treated group that exhibited no malformations (the ETU group) were detected by immunohistochemistry, western blot, and qRT-PCR. RESULTS: The fetal rats in the control group showed normal position of the anal opening, with no malformation. The incidence of ARM was 89.2% for the fetal rats in the experimental group. The immunohistochemistry results showed that P2Y2 was expressed in the cytoplasm of the cells in the terminal rectum submucosa and myenteric plexus of the fetus rats in the control group, the ETU group, and the ARM group. The average integrated optical density (IOD) value for the ARM group was significantly lower than the IOD value for the control and ETU groups (186.48 ± 23.03 vs. 493.18 ± 19.70; 186.48 ± 23.03 vs. 479.48 ± 41.71, P<0.01), while the IOD value for the ETU group was comparable to the control group IOD (493.18 ± 19.70 vs. 479.48 ± 41.71, P = 0.360). The western blot and qRT-PCR results showed that the P2Y2 protein and mRNA expressions were significantly lower in the terminal rectum of the fetal rats in the ARM group than in the control and ETU groups (0.28 ± 0.08 vs. 0.51 ± 0.10, 0.28 ± 0.08 vs. 0.48 ± 0.12; 48.91 ± 12.17 vs. 98.03 ± 15.68, 48.91 ± 12.17 vs. 92.53 ± 10.43; P<0.01), while the P2Y2 protein and mRNA levels in the control group were comparable to the ETU group (0.51 ± 0.10 vs. 0.48 ± 0.12, P = 0.494; 98.03 ± 15.68 vs. 92.53 ± 10.43, P = 0.058). CONCLUSION: P2Y2 may participate in and affect the development of ENS in the terminal rectum of fetal rats with ARM.

Bimetallic ruthenium complexes bridged by divinylphenylene bearing oligo(ethylene glycol)methylether: synthesis, (spectro)electrochemistry and the lithium cation effect.

A series of 1,4-disubstituted ruthenium-vinyl complexes, (E,E)-[{(PMe3)3(CO)ClRu}2(µ-HC=CH-Ar-CH=CH)], in which the 1,4-diethenylphenylene bridge bears two oligo(ethylene glycol)methyl ether side chains at different positions (2,5- and 2,3-positions), were prepared. The respective products were characterized by elemental analyses and NMR spectroscopy. The structures of complexes 1b and 1e were established by X-ray crystallography. The electronic properties of the complexes were investigated by cyclic voltammetry, and IR and UV-vis/NIR spectroscopies. Electrochemical studies showed that the 2,5-substituents better stabilized the mixed-valence states; the electrochemical behavior was greatly affected by lithium cations, especially complex 1g with 2,3-substituents, which was further supported by IR and UV-vis/NIR spectra changes. Spectroelectrochemical studies showed that the redox chemistry was dominated by the non-innocent character of the bridging fragment.

[Surgical correction of buried penis in children by solution and fixation of the penile venter].

OBJECTIVE: To search for a simple and effective surgical procedure for the treatment of the buried penis in children. METHODS: We designed a new surgical procedure for the treatment of the buried penis, making a "V" incision in the venter of the radix penis, solving the superficial and deep fascia and the abnormal attachment of the dartos muscle, and fixing the left and right side of the radix penis respectively to 1 and 11 o'clock points in the fascia of the pubis nodus in the knee-chest position. A total of 38 children with the buried penis underwent the newly designed procedure and were followed up for 2-6 months. RESULTS: All the patients were discharged with desirable cosmetic appearance of the penis and completely revealed penile shafts. Follow-up showed satisfactory penile shape in 35 cases (92.11%), slightly buried penis in 2 and cicatricial constriction of the ostium praeputiale in 1. CONCLUSION: The newly designed surgical procedure of solution and fixation of the penile venter is simple and effective for the treatment of the buried penis in children and well worthy to be introduced into clinical practice.