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A 19-year-old man with a history of Peutz-Jeghers syndrome (PJS) and two previous partial small bowel resections because of intussusception presented with lower abdominal pain. Computed tomography (CT) showed concentric multilayer and cord-like structures in the transverse colon. Colo-colonic intussusception was suspected and he was hospitalized. After two therapeutic enemas were unsuccessful, a colonoscopy was performed. The intussusception was reduced and a 40-mm transverse colon polyp with a thick stalk was resected. After the procedure, his abdominal pain was relieved and he was discharged on the sixth hospital day. This case and several previous reports suggest that PJS polyps with tumor diameter exceeding 30 mm and location in the transverse or sigmoid colon can cause intussusception. Endoscopic treatment should be considered for these lesions., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Purpose: Patients who developed apparent metachronous contralateral inguinal hernia (MCIH) after negative laparoscopic diagnosis have been reported. We performed this study to investigate the morphological characteristics and etiology of this phenomenon., Patients and Methods: A consecutive series of 1,747 patients (858 boys and 889 girls) with symptomatic unilateral inguinal hernia were studied. During laparoscopic percutaneous completely extraperitoneal closure, morphological appearances at the asymptomatic groin were inspected for contralateral patent processus vaginalis (CPPV) with definitive criteria. If positive CPPV was identified, it was closed by the same technique used for the affected side. The patients were reviewed for occurrence of metachronous contralateral hernia., Results: A total of 755 patients (43.2%) had positive CPPV. Of the 992 patients whose CPPVs were evaluated as true negative, eight (seven boys, one girl) developed MCIH (time taken, three months to five years six months). During second-look operations, morphological appearances of the CPPV that was originally considered as true negative CPPV was found to be wide open., Conclusions: Despite a true negative evaluation by laparoscopy, there was a 0.8% chance of developing a MCIH. This phenomenon was male-oriented and may be acquired indirect inguinal hernia without preceding of CPPV., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The Japanese Society of Emergency Pediatrics has formulated evidence-based guidelines for the management of intussusception in children in order to diagnose intussusceptions promptly, to initiate appropriate treatment as early as possible, and to protect intussuscepted children from death., Methods: Literature was collected systematically via the Internet using the key words "intussusception" and "children." The evidence level of each paper was rated in accordance with the levels of evidence of the Oxford Center for Evidence-based Medicine. The guidelines consisted of 50 clinical questions and the answers. Grades of recommendation were added to the procedures recommended on the basis of the strength of evidence levels., Results: Three criteria of "diagnostic criteria,""severity assessment criteria," and "criteria for patient transfer" were proposed aiming at an early diagnosis, selection of appropriate treatment, and patient transfer for referral to a tertiary hospital in severe cases. Barium is no longer recommended for enema reduction (recommendation D) because the patient becomes severely ill once perforation occurs. Use of other contrast media, such as water-soluble iodinated contrast, normal saline, or air, is recommended under either fluoroscopic or sonographic guidance. Delayed repeat enema improves reduction success rate, and is recommended if the initial enema partially reduced the intussusception and if the patient condition is stable., Conclusions: The guidelines offer standards of management, but it is not necessarily the purpose of the guidelines to regulate clinical practices. One should judge each individual clinical situation in accordance with experiences, available devices, and the patient's condition., (© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.)

Background: The Canadian Paediatric Triage and Acuity Scale (P-CTAS) is used and modified at hospitals as a triage tool for pediatric patients before they are seen in emergency rooms. Pediatric surgery patients account for very few of the many patients in emergency departments, but they should be triaged as emergency or urgent because they might be candidates for surgery. Problems with and improvements for triaging pediatric surgery patients using the P-CTAS were studied., Methods: This retrospective study evaluated all patients <16 years old who visited the emergency department of Kyorin University Hospital during an approximately 4 year period between 1 May 2005, and 11 February 2009. Pediatric surgery patients were divided into two groups to evaluate the efficiency of P-CTAS triage. Patients who needed emergency treatment were in group A, and the others were in group B., Results: Most group A patients were level I, II, or III (97%, 111/114). In contrast, 60% (71/119) of group B patients were level IV or greater. Some problems with and suggestions for the P-CTAS were identified. Many patients with trauma were under 1 year of age, and many with a foreign body were under 2 years of age. Age categories should be added for patients with trauma or foreign body aspiration. Patients with abdominal pain, and without anal bleeding or vomiting who are >2 years old are triaged as level IV and they accounted for 12% of patients with possible intussusception in this study. A category of 'possible intussusception' should be made for level II. Most patients with acute scrotum, whether operated on or not, were level III. 'Red or purple color of scrotal skin' and/or 'within 6 h from onset' could be added to level II for patients with acute scrotum., Conclusions: P-CTAS worked well for pediatric surgery patients, and it needs to be modified and improved for such patients based on these results., (© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.)

Background: Conventional open herniorrhaphy in children has been reported to have 0.3-3.8% recurrence and 5.6-30% postoperative contralateral hernia rates. We developed a unique technique to achieve completely extraperitoneal ligation of PPV without any skip areas under laparoscopic control. This report introduces our technique and results compared with the cut-down herniorrhaphy., Methods: A consecutive series of 1,585 children with inguinal hernia/hydrocele (1996-2006) was analyzed. In laparoscopic patent processus vaginalis (PPV) closure (LPC), an orifice of PPV was encircled with a 2-0 suture extraperitoneally by a specially devised Endoneedle and tied up from outside of the body achieving completely extraperitoneal ligation of the ring. The round ligament was included in the ligation, whereas the spermatic cord and testicular vessels were excluded by advancing the needle across them behind the peritoneum. Cut-down herniorrhaphy (CD), with or without diagnostic laparoscopy, or LPC was selected according to parental preference under informed consent., Results: Parents gave more preference to LPC (LPC in 1,257 children, CD in 308, and miscellaneous in 20). Age ranges were equal for both groups. Sex distribution showed female preponderance in the LPC group (44.8% vs. 26.6%, p < 0.001) and umbilical hernia/cysts were predominantly included in the LPC group (11.9% vs. 2.9%, p < 0.001). Mean operation times were equal for both groups for unilateral repair (28.2 +/- 9.2 for LPC vs. 27.8 +/- 13.5 for CD) and were shorter for bilateral repair in the LPC group (35.8 +/- 11.6 vs. 46.7 +/- 17.7). The incidence of postoperative hernia recurrence and contralateral hernia in the LPC group was 0.2% and 0.8%. Two children in the CD group had injuries to their reproductive system during the operation (0.6%)., Conclusions: The advantages of our technique include following: technically simple, short operation time, inspection of bilateral IIRs with simultaneous closure of cPPV, reproductive systems remain intact, routine addition of umbilicoplasty if desired, and essentially indiscernible wounds.

Teratoma in infants and children is not particularly rare. However, the teratoma in the hepatoduodenal ligament is extremely rare and the surgical treatment is challenging because of its anatomical complexity. We could find only six cases in the literature. In this report, we present the seventh case with special reference to the operative technique to save the hepatic artery for resection of the tumor using intraoperative ultrasonography.

Pyriform sinus fistula causes acute suppurative thyroiditis, and there is a risk of recurrence if the sinus tract is not excised completely. The tract should be dissected as high as possible toward the pyriform fossa. We report our devised technique "the light guided procedure" for the impalpable fistula. A bronchoscope was inserted into the pyriform sinus fistula with the help of a gastrofiberscope positioned at the larynx. The tract of the fistula was identified with the help of the light from the bronchoscope. We were able to reach the proximal end of the fistula, directly. This procedure is easy, safe, and minimally invasive.

Gastric teratomas are very rare and usually benign. Only a few cases of gastric teratomas with malignant components have been reported. This report describes recurrence of a yolk sac tumor following resection of a neonatal immature gastric teratoma. Gastric teratoma recurring as a malignant lesion has not been previously reported. Recurrence of immature gastric teratomas should be considered, and a periodic follow-up check with alpha-fetoprotein level should be mandatory.

A case of bronchopulmonary foregut malformation (BPFM) detected by multislice computed tomography with three-dimensional reconstruction (MSCT/3D) is reported. Concern for aspiration frequently limits the use of radiopaque contrast agents when anomalies of the lung and esophagus are suspected. MSCT/3D may make it possible to assess the communication and spatial relationship of malformed lung and esophagus in the early neonatal period without invasive or contrast radiological procedures such as bronchography or upper gastrointestinal series (UGI).

Protein-directed DNA bending is proposed to regulate assembly of higher-order DNA-multiprotein complexes (enhanceosomes and repressosomes). Because transcriptional initiation is a nonequilibrium process, gene expression may be modulated by the lifetime of such complexes. The human testis-determining factor SRY contains a specific DNA-bending motif, the high-mobility group (HMG) box, and is thus proposed to function as an architectural factor. Here, we test the hypothesis that the kinetic stability of a bent HMG box-DNA complex can in itself modulate transcriptional potency. Our studies employ a cotransfection assay in a mammalian gonadal cell line as a model for SRY-dependent transcriptional activation. Whereas sex-reversal mutations impair SRY-dependent gene expression, an activating substitution is identified that enhances SRY's potency by 4-fold. The substitution (I13F in the HMG box; fortuitously occurring in chimpanzees) affects the motif's cantilever side chain, which inserts between base pairs to disrupt base pairing. An aromatic F13 cantilever prolongs the lifetime of the DNA complex to an extent similar to its enhanced function. By contrast, equilibrium properties (specific DNA affinity, specificity, and bending; thermodynamic stability and cellular expression) are essentially unchanged. This correlation between potency and lifetime suggests a mechanism of kinetic control. We propose that a locked DNA bend enables multiple additional rounds of transcriptional initiation per promoter. This model predicts the occurrence of a novel class of clinical variants: bent but unlocked HMG box-DNA complexes with native affinity and decreased lifetime. Aromatic DNA-intercalating agents exhibit analogous kinetic control of transcriptional elongation whereby chemotherapeutic potencies correlate with drug-DNA dissociation rates.

Background/purpose: This report describes a technique devised for two children with megaduodenum consisting of subtotal duodenal resection with the proximal jejunum used as an onlay patch., Methods: A 2-year-old boy presented after unsuccessful surgical repairs for duodenal stenosis with web. Another 8-year-old boy returned from the United States after several surgical procedures for complicated congenital left diaphragmatic hernia with gastric outlet obstruction. Both boys manifested postprandial right upper abdominal fullness, occasional vomiting, abdominal pain, and failure to thrive. Upper gastrointestinal contrast study findings showed massively dilated duodenums in both patients that were larger than the stomach. During the operative procedures, about 95% of the duodenal wall was resected, leaving the basal portion that was unified with the pancreatic head and the ampulla of Vater as linguiform flap, which was covered with the proximal jejunum as an onlay patch so as to form the anterior wall of the duodenal bulb., Results: The patients showed markedly improved quality of life and catch-up growth after the operations. The duodenal bulbs were of adequate size in follow-up gastrointestinal series., Conclusion: This technique will be useful in cases of functional duodenal obstruction with megaduodenum secondary to congenital duodenal malformations leading to blind loop syndrome.

The high-mobility-group (HMG) box defines a DNA-bending motif conserved among architectural transcription factors. A "hydrophobic wedge" at the protein surface provides a mechanism of DNA bending: disruption of base stacking by insertion of a sidechain "cantilever." First described in the mammalian testis-determining factor SRY, the cantilever motif consists of adjacent aromatic and nonpolar sidechains at the crux of the HMG box (residues 12 and 13). Here, the role of these side chains in DNA recognition is investigated by alanine mutagenesis. F12A and I13A substitutions in the SRY HMG box each permit native folding and thermal stability (as monitored by circular dichroism and 1H-NMR) but eliminate sequence-specific DNA-binding activity (as detected by gel-mobility shift). On binding to the sharp angles of a four-way DNA junction (4WJ), however, the substitutions each promote formation of a high-molecular-weight aggregate, presumably by DNA-dependent oligomerization. The substitutions have opposite effects on initial binding to the 4WJ: whereas such binding is attenuated ten-fold by F12A, it is enhanced by I13A. A foreshortened "alanine cantilever", not observed among specific HMG boxes, occurs in a non-specific domain (HMG-1A) and may enhance architecture-selective DNA recognition.

SRY, a genetic "master switch" for male development in mammals, exhibits two biochemical activities: sequence-specific recognition of duplex DNA and sequence-independent binding to the sharp angles of four-way DNA junctions. Here, we distinguish between these activities by analysis of a mutant SRY associated with human sex reversal (46, XY female with pure gonadal dysgenesis). The substitution (168T in human SRY) alters a nonpolar side chain in the minor-groove DNA recognition alpha-helix of the HMG box [Haqq, C.M., King, C.-Y., Ukiyama, E., Haqq, T.N., Falsalfi, S., Donahoe, P.K., & Weiss, M.A. (1994) Science 266, 1494-1500]. The native (but not mutant) side chain inserts between specific base pairs in duplex DNA, interrupting base stacking at a site of induced DNA bending. Isotope-aided 1H-NMR spectroscopy demonstrates that analogous side-chain insertion occurs on binding of SRY to a four-way junction, establishing a shared mechanism of sequence- and structure-specific DNA binding. Although the mutant DNA-binding domain exhibits > 50-fold reduction in sequence-specific DNA recognition, near wild-type affinity for four-way junctions is retained. Our results (i) identify a shared SRY-DNA contact at a site of either induced or intrinsic DNA bending, (ii) demonstrate that this contact is not required to bind an intrinsically bent DNA target, and (iii) rationalize patterns of sequence conservation or diversity among HMG boxes. Clinical association of the I68T mutation with human sex reversal supports the hypothesis that specific DNA recognition by SRY is required for male sex determination.

A 7-year-old boy with giant cell fibroblastoma (GCF) of the skin and subcutaneous tissue of the right chest wall is described. To date, the histogenesis of GCF has not been clarified. The reason for the diversity of immunohistochemical data among various authors may be because the specimens studied were from only part of the lesion, or reduction of antigenicity through the preparation process. However, our findings based on studies of many specimens from various parts of the tumor for accurate immunohistochemical evaluation suggest that GCF may be a myofibrohistiocytic tumor. Recently, the suggestion that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP) has been reported. In addition to the present case, we performed immunohistochemical examination of 10 cases of definitely diagnosed DFSP for comparison. The immunohistochemical characteristics of these two neoplasms were concordant. However, from clinical and morphological viewpoints, it seems premature to recognize GCF as a juvenile form of DFSP.