Notes

Around the molecular, cell and also muscle origin of most cancers.
Biliary atresia (BA) is a devastating obstructive bile duct disease of newborns. BA has the highest incidence in Asians (1/5000), and its pathogenesis is unclear. We identified BA-private rare copy number variants (CNVs; 22 duplications and 6 deletions). ILF2 gene locates in the chromosome region (Chr1153410347-153,634,058) which was deleted in a nonsyndromic BA patient. However, it is still not known whether ILF2 plays a role in hepatobiliary development and its deletion impacts on the bile duct development.

To investigate if ILF2 is required for biliary development, we knock-out the zebrafish homologs of ILF2 by CRISPR/Cas9 approach, and discover that deletion of ILF2 causes a defective biliary development and a lack of bile flow from the liver to the gall bladder in zebrafish, which is a resemblance of phenotypes of BA.

Our data indicate that ILF2 gene is required for biliary development; deletion of ILF2 impairs bile duct development and could contribute to BA pathogenesis. This will be the first study to functionally evaluate the genes interfered by BA-private CNVs in hepatobiliary development and in BA pathogenesis.

Such functional study may reveal the potential value of these BA-private CNVs in the disease pathogenesis for BA.

N/A (animal and laboratory study).
N/A (animal and laboratory study).
Prophylactic, intraabdominal drains have been used to prevent intraabdominal abscess (IAA) after perforated appendicitis. We hypothesized that routine drain placement would reduce the IAA rate in pediatric perforated appendicitis.

A 27-month quality improvement (QI) initiative was conducted closed-suction, intraabdominal drains were placed intraoperatively in pediatric (age < 18) perforated appendicitis patients. QI patients were compared to controls admitted during the preceding 8 months and following 4 months. The primary outcome was 30-day IAA rate. Univariate and multivariate analyses were performed.

Two hundred seventy QI patients were compared to 109 controls. There was 100% compliance during 21 of 27 months of the QI initiative; only 7 QI patients did not receive drains. IAA occurred in 20.0% of QI patients and 22.9% of control (p = 0.52). After adjustment, the QI initiative was not associated with reduced odds of IAA (OR 0.83, 95% CI 0.48-1.44). Median length of stay was longer in QI patients during the index admission (p = 0.03) and over 30 postoperative days (p = 0.03), but these relationships did not persist after adjustment.

A QI initiative investigating prophylactic, intraabdominal drain placement in perforated appendicitis did not reduce the IAA rate. We recommend against routine drain placement in pediatric perforated appendicitis.

Level III.
Level III.
Pyriform sinus fistula (PSF) is a rare congenital anomaly, and the preferred definitive treatment is yet to be verified. In this study, we investigated the treatment outcomes of PSF specifically comparing endoscopic-assisted surgery and endoscopic radiofrequency ablation (RA).

The medical records of patients treated for PSF at the Shanghai Children's Hospital between October 2016 and September 2019 were retrospectively evaluated.

There were 93 girls and 98 boys. The median age at onset and operation was 3 years and 5 years, respectively. Endoscopic-assisted surgery was performed in 143 patients. During the same period, RA was performed in 48 patients, and 10 of them concurrently underwent incision and drainage of neck abscesses. Longer hospital stay was found in the endoscopic-assisted surgery group than in the RA group (10.50 ± 3.93 vs. 5.02 ± 3.30 days, P < 0.001). Postoperative complications were not significantly different between the two groups, except for neck infection (0 vs. HC-258 inhibitor 8.3%, P = 0.004). After a median follow-up period of 21 months, no significant difference was found between the two groups in terms of recurrence (1.4% vs. 0, P = 0.560).

Patients treated with RA had a significantly shorter hospital stay than those treated with endoscopic-assisted surgery. Outcomes of endoscopic-assisted surgery and RA were not significantly different for the management of PSF and treatment method should be tailored to the patient.

IV.
IV.
Intraluminal springs have recently been shown to lengthen segments of intestine in a process known as distraction enterogenesis. We hypothesized that biocompatible springs could be used to lengthen defunctionalized murine small intestine and would lead to identifiable intestinal adaptations at the molecular level.

Age and weight matched C57BL/6 mice underwent surgical insertion of nitinol spring-loaded capsules into a Roux limb of jejunum. Segment lengths were measured at initial spring placement and at euthanasia after 14 and 21 days. Histology and gene expression of the Roux limb were evaluated at scarification and compared to untreated control segments.

Intestinal segments loaded with compressed springs lengthened an average of 240%, which was significantly longer than control segments loaded with either empty capsules or uncompressed springs. Muscularis thickening was greater in spring-treated mice compared to controls without springs. Crypt depth and Lgr5+ expression was greater in mice that received compressed spring treatments when compared to control groups.

Insertion of a compressed nitinol spring into a Roux limb results in significant intestinal lengthening, smooth muscle thickening, and Lgr5+ expression in a mouse model. The ability to increase small bowel length in a defunctionalized murine model may be used to understand the mechanism of distraction enterogenesis.
Insertion of a compressed nitinol spring into a Roux limb results in significant intestinal lengthening, smooth muscle thickening, and Lgr5+ expression in a mouse model. The ability to increase small bowel length in a defunctionalized murine model may be used to understand the mechanism of distraction enterogenesis.Ankle arthrodesis (AA) and total ankle arthroplasty (TAA) are the 2 primary surgical treatments for patients with end-stage ankle arthritis. The comparative outcomes between AA and TAA using modern techniques remain unclear. A systematic search to identify all relevant articles comparing AA with TAA was conducted through 3 online databases. The clinical outcomes were extracted for meta-analysis, including AOFAS (American Orthopaedic Foot & Ankle Society) score, VAS (visual analog scale), AOS (Ankle Osteoarthritis Scale), gait analysis, ROM (range of motion), satisfaction, complication, and reoperation. Our meta-analysis shows no heterogeneity in any subgroup analyses. There were no significant differences in AOFAS total, pain, and alignment scores between the AA and TAA groups. The TAA group had significant improvement in AOFAS function score compared with the AA group. There was no significant difference in VAS and AOS total scores between the 2 groups. No significant differences in gait analysis were observed between the 2 groups.
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