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A 17-year-old boy presented with a high-grade fever. The patient had been previously diagnosed with Cornelia de Lange syndrome (CdLS). The patient visited a local physician and was diagnosed with sinusitis. Owing to persistent fever, the patient was referred to our hospital. At the initial presentation, his body temperature was 38.2°C, while maintaining previous living activities and neurological function. Despite changing the antibiotic to amoxicillin, the patient's fever persisted with worsened activity. At the second presentation, the patient presented with left hemiparesis. Blood examination revealed increased white blood cell count and serum C-reactive protein level. Emergency magnetic resonance imaging revealed acute cerebral infarcts in the right cerebral hemisphere, with evident stenosis in the right paraclinoid segment of the internal carotid artery (ICA). In addition, an abscess was found in the cerebellar hemisphere, which was punctured through the burr hole. Computed tomography performed after the completion of antibiotic therapy revealed a restored diameter of the stenotic ICA. Sinusitis can cause ischemic stroke due to compressive stenosis of the paraclinoid ICA, particularly in patients with CdLS. Sinusitis should be preferentially managed in patients with CdLS.

A 74-year-old man presented with persistent hiccups and headache persisting for 2 days. An anticoagulant was administered for his coronary heart disease. Cranial computed tomography (CT) revealed an intracerebral hemorrhage (ICH) located in the right occipital lobe, without any abnormal findings around the brainstem. The patient underwent endoscopic hematoma evacuation via a burr hole, resulting in immediate resolution of hiccups. Following an uneventful postoperative course, the patient experienced recurrent hiccups on the 47th day postsurgery. A subsequent CT scan taken on the 50th day revealed a compressive chronic subdural hematoma (CSDH) situated in the right frontoparietal convexity. The patient underwent burr-hole irrigation, leading to prompt cessation of the hiccups. Persistent hiccup should be recognized as potential manifestation of supratentorial lesions, including ICH or CSDH. Surgical evacuation of such lesions can rapidly alleviate hiccups associated with these pathologies.

A 50-year-old man presented with headache and left hemiparesis. No noticeable preceding head trauma was observed. Computed tomography (CT) scans revealed a compressive chronic subdural hematoma (CSDH). The patient underwent burr-hole irrigation, during which he was considerably restless. In addition, extensive avulsion was found in the parietal dura mater posterior to the burr hole. CT performed immediately after the surgery revealed the emergence of a thick epidural hematoma (EDH) located posterior to the burr-hole. During emergency craniotomy for the EDH, there was no identifiable injury to the dura mater or the meningeal vessels. However, a review of the CT scans confirmed well-developed diploic spaces just above the center of the EDH, with connecting channels between the diploic spaces and extracranial sites. Based on these observations, we assumed that the diploic vein might have caused the EDH. Diploic veins can cause AEDH after burr hole irrigation for CSDH. Appropriate intraoperative sedation and protective irrigation maneuvers can reduce the risk of such AEDH.

A 43-year-old, previously healthy man experienced a decreased sensation in the left lower extremity without preceding spinal trauma. At presentation, the patient exhibited slight motor weakness in the left lower extremity, in addition to decreased pain sensation below the ipsilateral T7. Spinal magnetic resonance imaging (MRI) revealed abnormal findings consistent with idiopathic thoracic spinal cord herniation (ITSCH) at the T5/6 level. Computed tomography (CT) revealed a small vertebral erosion at the lower T5. The patient's symptoms gradually progressed over the next 3 years. MRI revealed marked lateral elongation of the cord at the T5/6 and apparent intravertebral cord herniation. The patient underwent ITSCH reduction through T5-6 laminectomies. The herniated cord was vertically long with a bulbous rostral part. Successful ITSCH reduction was achieved and the patient's postoperative course was uneventful. ITSCH is a progressive pathology that requires prompt surgical reduction. Certain ITSCHs may be complicated by intravertebral cord herniation.

Abstract Development of subclassification of intermediate-stage hepatocellular carcinoma (HCC) by treatment suitability is in demand. We aimed to identify predictors that define treatment refractoriness against locoregional(transarterial chemoembolization(TACE) or thermal ablation) and surgical therapy. This multicenter retrospective study enrolled 1167 HCC patients between 2015 and 2021. Of those, 209 patients were initially diagnosed with intermediate-stage HCC. Treatment refractoriness was defined as clinical settings that meets the following untreatable progressive conditions by TACE (1) 25% increase of intrahepatic tumor, (2) transient deterioration to Child–Pugh class C, (3) macrovascular invasion or extrahepatic spread, within one year. We then analyzed factors contributing to treatment refractoriness. The Child–Pugh score/class, number of tumors, infiltrative radiological type, and recurrence were significant factors. Focusing on recurrence as a predictor, median time to untreatable progression (TTUP) was 17.2 months in the recurrence subgroup whereas 35.5 months in the initial occurrence subgroup (HR, 2.06; 95% CI, 1.44–2.96; P = 0.001). Median TTUP decreased in cases with more later times of recurrence (3–5 recurrences, 17.3 months; ≥ 6 recurrences, 7.7 months). Recurrence, even more at later times, leads to increased treatment refractoriness. Early introduction of multidisciplinary treatment should be considered against HCC patients after multiple recurrent episodes.

A 53-year-old woman presented with a 2-week history of headache and vertigo. Computed tomography revealed a hyperdense tumor, measuring 30 × 31 × 36 mm in diameter, in the anteromedial parts of the cerebellar hemispheres. Cerebral magnetic resonance imaging 10 days later revealed an apparent extra-axial tumor with broad attachment to the medial tentorium cerebelli and rapid growth to a diameter of 40 × 41 × 46 mm. Cerebral angiography revealed no obvious feeding vessels or tumor stains. The patient underwent biopsy through the left occipital transtentorial route. The histological appearance was consistent with diffuse large B-cell lymphoma. Intracranial lymphoma may present as a dural tumor that mimics a meningioma. Rapid tumor growth incongruous with benign meningiomas should be assumed to be possible lymphoma, and prompt biopsy should be performed.

Abstract Bile acids have received increasing attention as a marker of the long-term prognosis and a potential therapeutic target in patients with biliary atresia, which is a progressive disease of the hepatobiliary system. A detailed analysis of serum and urinary bile acid compositions was conducted to assess the characteristics of bile acid profiles and the correlation between bile acid profiles and liver fibrosis markers in adult patients with biliary atresia who achieved bilirubin normalization. Serum total bile acids and glucuronide-conjugated (glyco- and tauro-) cholic acids (GCA and TCA) and chenodeoxycholic acids (GCDCA and TCDCA) were significantly higher in patients with biliary atresia than in healthy controls, whereas unconjugated CA and CDCA showed no significant difference. There were no significant differences in CA to CDCA ratios and glycine-to-taurine-conjugated ratios. Urinary glycocholic acid 3-sulfate (GCA-3S) was significantly higher in patients with biliary atresia. Serum GCDCA showed a strong positive correlation with Mac-2 binding protein glycosylation isomer (M2BPGi). These results demonstrate that bile acid congestion persists into adulthood in patients with biliary atresia, even after cholestasis has completely improved after Kasai portoenterostomy. These fundamental data on bile acid profiles also suggest the potential value of investigating bile acid profiles in patients with biliary atresia.

Abstract Background Dysregulation of the terminal differentiation of bladder urothelium is associated with the pathogenesis of urinary tract disorders. Fibroblast growth factor (Fgf)7 and Fgf10 stimulate urothelial proliferation; however, their roles in cellular differentiation remain unclear. In this study, we used an organoid system to investigate the roles of these Fgfs in regulating bladder urothelium differentiation and identify their distribution patterns in the mouse bladder. Methods Adult bladder epithelia (AdBE) isolated from adult mouse bladder tissues (AdBTs) were used to culture adult bladder organoids (AdBOs) in the presence of Fgf7 and Fgf10. The differentiation status of the cells in AdBTs, AdBEs, AdBOs, and neonatal bladder tissues (NeoBTs) was analyzed via quantitative real-time-PCR for the presence of undifferentiated cell markers (Krt5, Trp63, and Krt14) and differentiated cell markers (Krt20, Upk1a, Upk2, and Upk3a). Organoid cell proliferation was assessed by counting cell numbers using the trypan blue method. The effects of Fgf7 and Fgf10 on organoid differentiation were assessed using different doses of Fgfs, and the involvement of peroxisome proliferator-activated receptor γ (PPARγ) signaling in these processes was tested by introducing a PPARγ agonist (Rosiglitazone) and antagonist (T0070907) to the culture. The expression patterns of Fgf7 and Fgf10 were examined via in situ hybridization of AdBTs. Results AdBOs showed higher expression of undifferentiated cell markers and lower expression of differentiated cell markers than AdBTs, NeoBTs, and AdBEs, indicating the relatively immature state of AdBOs. Differentiation of AdBOs was enhanced by Rosiglitazone and Fgf7, suggesting an interplay of intracellular signals between Fgf7 and PPARγ. Co-addition of T0070907 suppressed Fgf7-mediated differentiation, demonstrating that PPARγ is activated downstream of Fgf7 to promote cellular differentiation into umbrella cells. Furthermore, we found that Fgf7 is predominantly expressed in the umbrella cells of the urothelium, whereas Fgf10 is predominantly expressed in the urothelium and stroma of AdBTs. Conclusions We demonstrated that unlike Fgf10, Fgf7 induces cellular differentiation via PPARγ activity and has a unique tissue distribution pattern in the adult bladder. Further studies on the Fgf7-PPARγ signaling axis would provide insights into the differentiation mechanisms toward functional umbrella cells and the pathogenesis of several urinary tract diseases.

Background Following on from an earlier study published in 2008 about left pulmonary artery (LPA) flow measured on serial echocardiography being strongly prognostic in left-sided congenital diaphragmatic hernia (CDH) and the ratio of LPA to right pulmonary artery (RPA) diameters being a simple and reliable indicator for commencing nitric oxide (NO) therapy, the ratio of LPA:RPA diameters (PA ratio or PAR) was hypothesized to possibly reflect cardiopulmonary stresses accompanying CDH better.Methods Subjects with isolated left-sided CDH treated between 2007 and 2020 at a single pediatric surgical center were recruited and classified according to survival. Data obtained retrospectively for subject demographics, clinical course, LPA/RPA diameters, and PAR were compared between survivors and non-survivors. The value of PAR for optimizing the prognostic value of PA diameter data in CDH were analyzed with receiver operating characteristic (ROC) curve analysis.Results Of 65 subjects, there were 54 survivors (82.3%) and 11 non-survivors (17.7%); 7 of 11 non-survivors died before surgical repair could be performed. Mean PAR for survivors (0.851±0.152) was significantly higher than for non-survivors (0.672±0.108) (p=0.0003). Mean PAR for non-survivors was not affected by surgical repair. Characteristics of survivors were: LPA ≥2 mm (n=52 of 54; mean PAR=0.866±0.146) and RPA ≥3 mm (n=46 of 54; mean PAR=0.857±0.152). Non-survivors with similar LPA and RPA diameters to survivors had significantly lower mean PAR. ROC curve cut-off for PAR was 0.762. Subjects with high PAR (≥0.762) required high-frequency oscillatory ventilation/NO less than subjects with low PAR (

ObjectiveThe aim of the study is to discuss the efficacy of live vs. remote cadaver surgical training (CST) for minimally invasive surgery (MIS).MethodsA cohort of 30 interns in their first and second years of training were divided into three groups: live observers (n = 12), live participants (n = 6), and remote observers: (n = 12). The interns had the opportunity to either observe or actively participate in two different surgical procedures, namely, laparoscopic lower anterior resection, performed by a colorectal surgical team, and laparoscopic fundoplication, performed by a pediatric surgical team. The procedures were conducted either at a base center or at a remote center affiliated with the institute. Some of the interns interacted directly with the surgical teams at the base center, and others interacted indirectly with the surgical teams from the remote center. All interns were administered questionnaires before and after completion of the CST in order to assess their understanding of various aspects related to the operating room layout/instruments (called “design”), accessing the surgical field (called “field”), understanding of anatomic relations (called “anatomy”), their skill of dissection (called “dissection”), ability to resolve procedural/technical problems (called “troubleshooting”), and their skill in planning surgery (called “planning”) according to their confidence to operate using the following scale: 1 = not confident to operate independently; 4 = confident to operate with a more senior trainee; 7 = confident to operate with a peer; and 10 = confident to operate with a less experienced trainee. A p

AimsEarly postoperative outcome (EPO) was compared between fully laparoscopic Duhamel-Z (F-Dz) and laparoscopy-assisted Duhamel-Z (A-Dz) anastomoses performed for total colonic aganglionosis (TCA).MethodsEPO was assessed quarterly for the first year after F-Dz/A-Dz using a continence evaluation score (CES) based on stool frequency (motions/day) and stool consistency (0 = liquid, 1 = soft, 2 = formed), presence of anal erosion (0 = severe, 1 = moderate, 2 = mild), and incidence of enterocolitis.Surgical technique involved taking the ileostomy down, dissecting the colon laparoscopically, and preparing the pull-through ileum through the stoma wound. In F-Dz (n = 3), a working port (SILS trocar) was inserted, and laparoscopic retrorectal dissection with forceps used to create a retrorectal tunnel from the peritoneal reflection extending downward as narrow as possible along the posterior wall of the rectum to prevent lateral nerve injury and preserve vascularity. After completing the tunnel, the ileum was pulled-through from an incision on the anorectal line and a Z-shaped ileorectal side-to-side anastomosis performed without a blind pouch. In A-Dz (n = 11), the retrorectal pull-through route was created through a Pfannenstiel incision using blunt manual (finger) dissection along the anterior surface of the sacrum.ResultsSubject backgrounds were similar. Mean quarterly data were: frequency (F-Dz: 4.67, 4.67, 4.67, 3.33) vs. (A-Dz: 7.27, 7.09, 6.18, 5.36) p