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The effectiveness of a hybrid closed-loop (HCL) system in improving glycemic control is unclear in Japanese individuals. Therefore, we assessed the effect impact of the MiniMed 770G HCL system on glycemic control in this population. This prospective, single-center, 24-week observational study (registration number: UMIN000047394) enrolled 23 individuals with type 1 diabetes mellitus using the Medtronic MiniMed 640G system. The primary endpoint was the improvement in time in the range of 70-180 mg/dL after transitioning to the MiniMed 770G HCL system. We observed an increase in time in range (from 64.1 [55.8-69.5] to 70.9 [67.1-74.4] %, interquartile range 25-75%, p < 0.001) and a decrease in glycated hemoglobin level (from 7.4 [7.0-7.9] to 7.1 [6.8-7.4] %, p = 0.003). There was a significant reduction in time above the range (181-250 mg/dL: 25.8 [20.9-28.6] to 19.5 [17.1-22.1] %, p < 0.001; >251 mg/dL: 8.7 [4.0-13.0] to 4.7 [3.6-9.1] %, p < 0.001). Time below the range remained unchanged (54-69 mg/dL: 1.8 [0.4-2.4] to 2.1 [0.4-3.9] %, p = 0.24; <54 mg/dL: 0.2 [0.0-1.0] to 0.5 [0.1-1.3] %, p = 0.14). In a subgroup of 12 patients with a high HCL implementation rate, the basal insulin infusion decreased immediately after mealtime insulin administration and increased after approximately 120 minutes. The ratings from questionnaires assessing treatment burden, satisfaction, and quality of life remained unchanged. The MiniMed 770G HCL system improved glycemic control and optimized insulin delivery, particularly in patients with high implementation rates.
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BACKGROUND: Long-term urinary outcomes after anorectal malformation (ARM) repair are affected by surgical approach and sacral anomalies. This study aimed to compare laparoscopic-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) in terms of urinary complications. METHODS: Between 2001 and 2022, 45 patients were treated with LAARP or PSARP. The rectourethral fistula and inflow angle between the fistula and rectum was confirmed by preoperative colonography. The incidence of urinary complications and treatment were compared between the two groups. RESULTS: Four patients (14%) had remnant fistula and five patients (17%) had neurogenic bladder dysfunction in LAARP group, while three patients (18%) had urethral injury in PSARP group. All patients with remnant fistula were asymptomatic and followed without treatment. The incidence of remnant fistula improved between earlier decade and later decade. In all cases with urethral injury, suture repair was performed and no postoperative leakage was noted. All five patients with neurogenic bladder dysfunction had spine abnormalities that required clean intermittent catheterization (CIC) and two were free from CIC finally. CONCLUSIONS: It is important to check inflow angle preoperatively to prevent remnant fistula. For PSARP, meticulous dissection is required when separating fistula from urethra because they create common wall. The most contributing factor to neurogenic bladder is sacral anomalies. Preoperative evaluation and postoperative urinary drainage are important.
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Malformaciones Anorrectales , Laparoscopía , Fístula Rectal , Enfermedades Uretrales , Vejiga Urinaria Neurogénica , Fístula Urinaria , Humanos , Lactante , Recto/cirugía , Recto/anomalías , Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/epidemiología , Vejiga Urinaria Neurogénica/etiología , Laparoscopía/efectos adversos , Resultado del Tratamiento , Fístula Rectal/cirugía , Fístula Rectal/complicaciones , Fístula Urinaria/etiología , Fístula Urinaria/cirugía , Enfermedades Uretrales/etiología , Enfermedades Uretrales/cirugía , Complicaciones Posoperatorias/etiología , Uretra/cirugía , Estudios Retrospectivos , Canal Anal/anomalíasRESUMEN
BACKGROUND: We previously showed that total tumor resection enhances metastatic growth in a syngeneic metastatic mouse model of neuroblastoma. In this study, we further investigated which surgical factors contributed most to metastatic growth. METHODS: Tumor cells derived from MYCN transgenic mice were subcutaneously injected into wild-type mice. Mice were randomly assigned to receive partial resection (PR group), subcutaneous implantation of a sponge (Sp group), or observation (Obs group). The lymph node metastasis volume and the frequency of lung metastasis were compared 14 days after assignment by measuring C-reactive protein (CRP) and interleukin-6 (IL-6) levels. RESULTS: The lymph node metastasis volume in the Sp group was larger than in the Obs group (148.4 [standard deviation {SD}: 209.5] vs. 10.2 [SD 12.8] mm3). The frequency of lung metastasis was greater in the Sp group than in the PR group (11.9 [SD 12.2] vs. 6.6 [SD 4.0] counts/slide). The CRP level in the Sp group was higher than in the PR group (2.3 [SD 0.5] vs. 1.5 [SD 0.4] µg/mL), and the IL-6 level in the Sp group was higher than in the PR or Obs groups (28.4 [SD 34.5] vs. 12.4 [SD 19.0] vs. 5.4 [SD 8.1] pg/mL). CONCLUSION: Metastatic growth may be enhanced by systemic inflammation.
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Proteína C-Reactiva , Modelos Animales de Enfermedad , Inflamación , Neoplasias Pulmonares , Neuroblastoma , Animales , Neuroblastoma/patología , Ratones , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/secundario , Proteína C-Reactiva/metabolismo , Inflamación/patología , Interleucina-6 , Metástasis Linfática , Ratones TransgénicosRESUMEN
BACKGROUND: Intrahepatic bile duct (IHBD) stones are one of the most common late complications of Roux-en-Y hepaticojejunostomy for congenital biliary dilatation (CBD). We report the current treatment strategies for IHBD stones and their outcomes in our institute. METHODS: Between 1983 and 2021, 117 patients with CBD were surgically treated in our institute. Our treatment strategies included oral ursodeoxycholic acid (UDCA), double-balloon endoscopic retrograde cholangiography (DB-ERC), percutaneous cholangio-drainage (PTCD), and open surgery. A retrospective study was conducted using medical charts. RESULTS: Postoperative IHBD stones were identified in 12 of 117 patients with CBD (10.2%). Five patients received UDCA, and small stones were successfully resolved in two cases. DB-ERC was performed eight times in five patients, but the endoscope could not reach the porta hepatis due to a long jejunal loop in two of five patients. One patient presented with severe acute pancreatitis induced by prolonged DB-ERC. PTCD was performed in three patients, two of whom finally underwent open surgery due to unsuccessful lithotomy. Open surgery was eventually performed in three patients. Lithotomy was performed in one patient; lithotomy with strictureplasty was performed in another patient. The other patient was diagnosed with intrahepatic cholelithiasis with adenocarcinoma. He underwent left lobectomy and died of carcinomatous peritonitis. CONCLUSIONS: Oral UDCA may be effective for small stones. Although DB-ERC should be considered as a first-line interventional therapy for lithotomy, it may not be feasible due to a long jejunal loop, and pancreatitis may occur. Long-term follow-up and early detection and treatment for IHBD stones may yield a good prognosis.
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Quiste del Colédoco , Pancreatitis , Masculino , Humanos , Estudios Retrospectivos , Enfermedad Aguda , Conductos Biliares Intrahepáticos/cirugía , Ácido UrsodesoxicólicoRESUMEN
BACKGROUND: The leading pathology of biliary atresia (BA) is inflammatory and fibrous obstruction of extrahepatic bile duct, but the pathogenesis remains unclear. IL13 is a cytokine associated with allergies and inflammatory fibrosis, and periostin induces fibrogenesis by stimulation with IL13. We analyzed the involvement of IL13 and periostin in inflammatory fibrosis in the extrahepatic bile duct of BA patients. MATERIALS AND METHODS: Surgically resected tissues from the hepatic hilar area of BA patients were immunostained with CD45, α-SMA, IL13 and periostin and statistically analyzed. Fibroblasts from the resected tissue were cultured with recombinant IL13, and periostin production was analyzed by quantitative polymerase chain reaction and Western blotting. RESULTS: IL13 was stained in 93% of large and micro bile ducts, and 92.1% matched with the CD45 location (p = 0.006) around the large bile ducts. Periostin staining correlated with the localization of IL13 and αSMA (p < 0.001) around the large bile ducts. Periostin mRNA and protein were upregulated by IL13 stimulation in cultured fibroblasts. CONCLUSION: IL13 was associated with induced periostin expression by fibroblasts, playing a vital role in the pathogenesis of fibrogenesis around the extrahepatic bile duct in BA.
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Conductos Biliares Extrahepáticos , Atresia Biliar , Moléculas de Adhesión Celular , Interleucina-13 , Humanos , Conductos Biliares/cirugía , Conductos Biliares/patología , Atresia Biliar/cirugía , Atresia Biliar/metabolismo , Fibrosis , Interleucina-13/metabolismo , Hígado/metabolismo , Moléculas de Adhesión Celular/metabolismoRESUMEN
PURPOSE: We aimed to evaluate the effect of human mesenchymal stem cells (hMSCs) on congenital diaphragmatic hernia (CDH) by intra-amniotic injection in a rat CDH model. METHODS: Nitrofen (100 mg) was administered to pregnant rats at E9.5. hMSCs (1.0 × 106) or PBS was injected into each amniotic cavity at E18, and fetuses were harvested at E21. The fetal lungs were classified into normal, CDH, and CDH-hMSCs groups. To determine the lung maturity, we assessed the alveolar histological structure by H&E and Weigert staining and the alveolar arteries by Elastica Van Gieson (EVG) staining. TTF-1, a marker of type II alveolar epithelial cells, was also evaluated by immunohistochemical staining and real-time reverse transcription polymerase chain reaction. RESULTS: The survival rate after intra-amniotic injection was 72.1%. The CDH-hMSCs group had significantly more alveoli and secondary septa than the CDH group (p < 0.05). The CDH-hMSCs group had larger air spaces and thinner alveolar walls than the CDH group (p < 0.05). The medial and adventitial thickness of the pulmonary artery in the CDH-hMSCs group were significantly better (p < 0.001), and there were significantly fewer TTF-1-positive cells than in the CDH group (p < 0.001). CONCLUSION: These results suggest that intra-amniotic injection of hMSCs has therapeutic potential for CDH.
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Hernias Diafragmáticas Congénitas/embriología , Hernias Diafragmáticas Congénitas/terapia , Células Madre Mesenquimatosas , Amnios , Animales , Modelos Animales de Enfermedad , Femenino , Humanos , Inyecciones , Pulmón/embriología , Embarazo , Ratas , Ratas Sprague-DawleyRESUMEN
Mesenchymal stem cells (MSCs) isolated from spinal ligaments with ectopic ossification have a propensity toward the osteogenic lineage. To explore epigenetic control of the osteogenic features of MSCs, we treated MSCs obtained from the spinal ligaments of ossification of yellow ligament (OYL) patients and non-OYL patients with the DNA methyltransferase inhibitor, 5-aza-2'-deoxycytidine (5AdC). We compared the non-OYL groups (untreated and treated with 5AdC) with the OYL groups (untreated and treated with 5AdC) by genome-wide microarray analysis. Next, we used methylated DNA immunoprecipitation combined with quantitative real-time PCR to assess gene methylation. Ninety-eight genes showed expression significantly increased by 5AdC treatment in MSCs from non-OYL patients but not from OYL patients. In contrast, only two genes, GDNF and WNT5A, showed significantly higher expression in OYL MSCs compared with non-OYL MSCs without 5AdC treatment. Both genes were hypermethylated in non-OYL MSCs but not in OYL MSCs. Small interfering RNA targeted to each gene decreased expression of the target gene and also several osteogenic genes. Both small interfering RNAs also suppressed the activity of alkaline phosphatase, a typical marker of osteogenesis. These results suggest that the osteogenic features of MSCs from OYL patients are promoted by unmethylated WNT5A and GDNF genes.
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Metilación de ADN , Factor Neurotrófico Derivado de la Línea Celular Glial/genética , Células Madre Mesenquimatosas/patología , Osificación Heterotópica/genética , Regiones Promotoras Genéticas , Proteínas Proto-Oncogénicas/genética , Proteínas Wnt/genética , Anciano , Anciano de 80 o más Años , Células Cultivadas , Femenino , Humanos , Ligamentos/citología , Masculino , Persona de Mediana Edad , Osificación del Ligamento Longitudinal Posterior/genética , Columna Vertebral , Análisis de Matrices Tisulares , Proteína Wnt-5aRESUMEN
BACKGROUND: Tumor recurrence, anorectal and urinary dysfunction, and lower limb dysfunction after surgery are observed in infantile sacrococcygeal teratoma (SCT). In this paper, a multi-institutional retrospective observational study was conducted to clarify the long-term functional prognosis in Japan. METHODS: This study was conducted using a paper-based questionnaire distributed to 192 facilities accredited by the Japanese Society of Pediatric Surgeons, covering patients who underwent radical surgery at less than 1 year old and who survived for at least 180 days after birth from 2000 to 2019. RESULTS: A total of 355 patients were included in this analysis. Altman type was I-II in 248 and type III-IV in 107, and the median maximum tumor diameter was 6.1 (range: 0.6-36.0) cm. There were 269 mature teratomas, 69 immature teratomas, and 10 malignant tumors. Total resection was performed in 325, subtotal or partial resection in 27, and surgical complications were noted in 54. The median postoperative follow-up was 6.6 (0.5-21.7) years. Eighty-three patients (23.4 %) had functional sequelae, including 62 (17.5 %) with anorectal dysfunction, 56 (13.0 %) with urinary dysfunction, and 15 (4.2 %) with lower limb motor dysfunction. Recurrence occurred in 42 (11.8 %) at a median age of 16.8 (1.7-145.1) months old. Risk factors for dysfunction included preterm delivery, a large tumor diameter, Altman type III-IV, incomplete resection, and surgical complications. Risk factors for recurrence included immature teratoma or malignancy, incomplete resection, and surgical complications. CONCLUSIONS: Postoperative dysfunction was not low at 23.4 %, and 11.8 % of the patients experienced recurrence occurring more than 10 years after surgery, suggesting the need for periodic imaging and tumor markers evaluations in patients with risk factors. It is necessary to establish treatment guidelines for best practice monitoring of the long-term quality of life. LEVEL OF EVIDENCE: Level II Retrospective Study.
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Neoplasias Pélvicas , Neoplasias de la Columna Vertebral , Teratoma , Niño , Humanos , Lactante , Japón/epidemiología , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/patología , Neoplasias Pélvicas/epidemiología , Neoplasias Pélvicas/cirugía , Calidad de Vida , Estudios Retrospectivos , Región Sacrococcígea/patología , Neoplasias de la Columna Vertebral/patología , Teratoma/epidemiología , Teratoma/cirugía , Teratoma/complicaciones , Preescolar , Adolescente , Adulto Joven , AdultoRESUMEN
A 56-year-old man presented with a history of hypertension; clinically, the patient had primary aldosteronism (PA) and a 4-cm left adrenal tumor. The left adrenal glands, resected by adrenalectomy, also contained ectopic thyroid tissue (ETT). An immunohistochemical analysis of steroid-converting enzymes revealed an aldosterone-producing adenoma (APA). Among 19 previously reported cases of adrenal ETT, 4 had adrenal hormonal abnormalities, all of which were PA. This is the first case of adrenal ETT coexisting with APA, confirmed by steroid-converting enzyme expression. Further analyses using cumulative case data are required to clarify the correlation between adrenal ETT and APA.
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Neoplasias de la Corteza Suprarrenal , Adenoma Corticosuprarrenal , Hiperaldosteronismo , Disgenesias Tiroideas , Masculino , Humanos , Persona de Mediana Edad , Adenoma Corticosuprarrenal/complicaciones , Adenoma Corticosuprarrenal/diagnóstico , Adenoma Corticosuprarrenal/cirugía , Aldosterona , Hiperaldosteronismo/complicaciones , Hiperaldosteronismo/diagnóstico , Glándulas Suprarrenales/metabolismo , Adrenalectomía , Disgenesias Tiroideas/complicaciones , Neoplasias de la Corteza Suprarrenal/complicaciones , Neoplasias de la Corteza Suprarrenal/diagnóstico , Neoplasias de la Corteza Suprarrenal/cirugíaRESUMEN
We encountered a 27-year-old Japanese woman with sensorineural deafness progressing to motor and sensory neuropathy. At 16 years old, she had developed weakness in her lower extremities and hearing impairment, which gradually deteriorated. At 22 years old, combined audiological, electrophysiological, and radiological examination results were consistent with auditory neuropathy spectrum disorder (ANSD). Genetic analyses identified a previously reported missense variant in the ATP1A1 gene (NM_000701.8:c.1799C>G, p.Pro600Arg). Although sensorineural deafness has been reported as a clinical manifestation of ATP1A1-related disorders, our case suggested that ANSD may underlie the pathogenesis of deafness in ATP1A1-related disorders. This case report broadens the genotype-phenotype spectrum of ATP1A1-related disorders.
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Enfermedad de Charcot-Marie-Tooth , Sordera , Pérdida Auditiva Central , Pérdida Auditiva Sensorineural , Femenino , Humanos , Adolescente , Adulto Joven , Adulto , Pérdida Auditiva Central/diagnóstico , Pérdida Auditiva Central/genética , Pérdida Auditiva Central/complicaciones , Pérdida Auditiva Sensorineural/diagnóstico , Pérdida Auditiva Sensorineural/genética , Enfermedad de Charcot-Marie-Tooth/genética , Sordera/complicaciones , ATPasa Intercambiadora de Sodio-PotasioRESUMEN
Aim: To clarify the characteristics of patients with rectal prolapse after laparoscopically assisted anorectoplasty (LAARP), estimate the causes, and evaluate its impact on postoperative bowel function. Methods: The medical records of patients who underwent LAARP for high- or intermediate-type anorectal malformation between 2000 and 2019 were retrospectively reviewed. Clinical data were compared between patients with (Group P) and without prolapse (normal, Group N). Fecal continence was evaluated using the clinical assessment score for fecal continence developed by the Japanese Study Group of Anorectal Anomalies. For patients who underwent pelvic magnetic resonance imaging (MRI) before LAARP, atrophy, or asymmetry of the anal sphincter and levator ani was evaluated by a radiologist. Results: Of the 49 patients, 29 (59%) had rectal prolapse after LAARP (Group P) and 20 did not (Group N). We found no significant difference in gender, type of malformations, incidence of associated spinal or lumbosacral anomalies, procedure time, and postoperative bowel function at ages 4, 8, 12, and 16 years. However, LAARP was performed significantly earlier in Group N (median [range], 180 [123-498] days) than in Group P (210 [141-570] days). In Group P, 18 patients (62%) developed prolapse before colostomy takedown. Eight of 26 patients who underwent surgical prolapse repair required redo procedures. Twenty-five patients who underwent preoperative pelvic MRI showed no significant relationship between the muscular abnormalities and the incidence of postoperative rectal prolapse. Conclusions: Although recurrence is common, performing LAARP at a younger age might prevent postoperative prolapse development.
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Malformaciones Anorrectales/cirugía , Laparoscopía/métodos , Prolapso Rectal/cirugía , Recto/cirugía , Adolescente , Malformaciones Anorrectales/fisiopatología , Niño , Preescolar , Defecación , Femenino , Humanos , Masculino , Periodo Posoperatorio , Prolapso Rectal/fisiopatología , Recto/anomalías , Estudios RetrospectivosRESUMEN
BACKGROUND: Pulmonary hypoplasia is an important cause of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). This study aimed to verify our hypothesis that the abnormal development of bronchial cartilage as well as alveolar immaturity, might play a central role in hypoplasia of the lung in human CDH. METHOD: We retrospectively analyzed autopsied lungs from 10 CDH cases and compared with nine age-matched controls to assess the bronchial cartilage and alveolar maturity using morphological techniques. RESULT: Ki-67 and thyroid transcription factor-1 (TTF-1) expression in the alveoli significantly increased in bilateral lungs with CDH. The shortest distance from the bronchial cartilage to the pleura was significantly shorter in ipsilateral (left) lungs with CDH, showing a positive correlation with the radial alveolar count (RAC). Regarding the small bronchial cartilages less than 20 000 µm2 , the average cartilage area significantly decreased in left lungs with CDH, and tended to decrease in right lungs with CDH. In addition, cartilage around the bronchi less than 200 µm in diameter tended to be smaller in left lungs with CDH. In contrast, regarding the cartilage around the bronchi 200 to 400 µm in diameter, the ratio of the total cartilage area relative to the bronchial diameter tended to be higher in left lungs with CDH, although there was a large variation. CONCLUSIONS: These opposite directional cartilage abnormalities around the distal and more proximal bronchi support our hypothesis that abnormal development of bronchial cartilage might play an important role in the hypoplastic lung in CDH.
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Bronquios/anomalías , Cartílago/anomalías , Hernias Diafragmáticas Congénitas , Femenino , Hernias Diafragmáticas Congénitas/metabolismo , Humanos , Recién Nacido , Antígeno Ki-67/metabolismo , Masculino , Alveolos Pulmonares/metabolismo , Estudios Retrospectivos , Factor Nuclear Tiroideo 1/metabolismoRESUMEN
A 32-year-old woman was referred to our hospital because of severe psychosis and was found to have an ectopic ACTH-producing thymic neuroendocrine tumor. Laboratory data revealed an elevated serum cortisol and plasma ACTH level, hypokalemia, and metabolic alkalosis. Chest computed tomography (CT) revealed an anterior mediastinal mass and multiple pulmonary nodules. As the patient was unable to communicate because of her consciousness disturbance, she was managed with artificial ventilation and deep sedation. Metyrapone and potassium supplementation were administered, and steroid psychosis gradually improved. Thoracic surgery was performed and the histopathological diagnosis was thymic neuroendocrine tumor with positive anti-ACTH immunohistochemical staining. Here we present details of the case and review the literature.