Purpose: The study reviewed a multidisciplinary approach to treating cervicofacial lymphatic malformations (CFLMs) in children.
Methods: Between 2007 and 2023, 53 children with CFLMs were treated with the median on-set age of 5 months (0-165) at our institute. For infants, airway management, including possible tracheotomy was prioritized, and a "wait-and-see" policy was adopted to expect spontaneous regression.
We herein report two- (2D) and three-dimensional (3D) culture methods of cholangiocytes originating from extrahepatic bile ducts of biliary atresia (BA) patients. Cells were stabilized for in vitro analyses, and 3D culture by two different methods showed the structural and functional features of cholangiocytes in the gel scaffold. First, cells were obtained from gallbladder contents or resected tissues of patients at surgery and then cultured in our original conditioned medium with a cocktail of signaling inhibitors that maintains the immaturity and amplification of cells.
View Article and Find Full Text PDFIntroduction: Biliary atresia (BA) is a cholestatic hepatopathy caused by fibrosing destruction of intrahepatic and extrahepatic bile ducts, and its etiology has not been clearly revealed. In BA, liver fibrosis progression is often observed even after Kasai portoenterostomy (KPE), and more than half of cases require liver transplantation in their lifetime in Japan. Macrophages play an important role in liver fibrosis progression and are classically divided into proinflammatory (M1) and fibrotic macrophages (M2), whose phenotypic transformation is called "macrophage polarity.
View Article and Find Full Text PDFBackground: Clinical trials have reported the efficacy of immune checkpoint inhibitors in the treatment of mismatch repair-deficient (dMMR) advanced solid tumors. The accumulated evidence of tumor agnostic agent has been made since PD-1 inhibitor was approved and used in clinical practice. Therefore, we have revised the guideline "Japan Society of Clinical Oncology provisional clinical opinion for the diagnosis and use of immunotherapy in patients with deficient DNA mismatch repair tumors, cooperated by Japanese Society of Medical Oncology, First Edition".
View Article and Find Full Text PDFThe development of novel antitumor agents and accompanying biomarkers has improved survival across several tumor types. Previously, we developed recommendations for tumor-agnostic treatments in patients with solid tumors with DNA mismatch repair deficient or neurotrophic receptor tyrosine kinase fusions. Recently, immune checkpoint inhibitors have shown efficacy in patient with tumor mutation burden-high (TMB-H) solid tumors and have been established as a third tumor-agnostic agent, making it necessary to develop the guideline prioritized for these patients.
View Article and Find Full Text PDFBackground: Clinical trials have reported the efficacy of tropomyosin receptor kinase (TRK) inhibitors against neurotrophic receptor tyrosine kinase (NTRK) fusion gene-positive advanced solid tumors. The accumulated evidence of tumor-agnostic agent has made since TRK inhibitors were approved and used in clinical practice. Therefore, we have revised the 'Japan Society of Clinical Oncology (JSCO)/Japanese Society of Medical Oncology (JSMO)-led clinical recommendations on the diagnosis and use of tropomyosin receptor kinase inhibitors in adult and pediatric patients with neurotrophic receptor tyrosine kinase fusion-positive advanced solid tumors, cooperated by the Japanese Society of Pediatric Hematology/Oncology (JSPHO)'.
View Article and Find Full Text PDFTrue thymic hyperplasia is defined as an increase in both the size and weight of the gland, while maintaining a normal microscopic architecture. Massive true thymic hyperplasia is a rare type of hyperplasia that compresses adjacent structures and causes various symptoms. Limited reports address the imaging findings of massive true thymic hyperplasia.
View Article and Find Full Text PDFSemin Pediatr Surg
February 2021
Reflections of academic pediatric surgery in Japan are shared by the authors. As in most areas of surgical practice committement and life long dedication are emphasized as the key(s) to success. An enquiring mind is always an advantage.
View Article and Find Full Text PDFIntroduction: Metachromatic leukodystrophy (MLD) refers to leukodystrophy caused by the accumulation of sulfatide from arylsulfatase A (ARSA) gene mutations. Sulfatide also accumulates in various organs, including the peripheral nerves, kidney, and gallbladder. Proliferative changes in the gallbladder have been reported in several patients, while gallbladder cancer is reported in only two adult MLD cases.
View Article and Find Full Text PDFBackground: The development of novel antitumor agents and accompanying biomarkers has improved survival across several tumor types. Previously, we published provisional clinical opinion for the diagnosis and use of immunotherapy in patients with deficient DNA mismatch repair tumors. Recently, efficacy of tropomyosin receptor kinase inhibitors against neurotrophic receptor tyrosine kinase (NTRK) fusion gene-positive advanced solid tumors have been established as the second tumor-agnostic treatment, making it necessary to develop the guideline prioritized for these patients.
View Article and Find Full Text PDFA 9-year-old girl was diagnosed with primary alveolar soft part sarcoma of the bladder after imaging examinations and transurethral resection (TUR) of the bladder tumor. As a positive surgical margin of the TUR indicated residual tumor cells, we performed a cystourethrectomy to remove the tumor. A continent urinary reservoir for self-catheterization was constructed using the Mainz pouch technique, and an abdominal (umbilical) continent catheterizable stoma using the appendix was performed.
View Article and Find Full Text PDFRationale: Liver heterotopia associated with congenital diaphragmatic hernia (CDH) is a rare condition; to the best of our knowledge, only 17 cases have been reported to date. The histogenesis and clinicopathological features are largely unknown. We herein report 2 cases of liver heterotopia associated with CDH along with 17 cases described in the literature to shed light on their clinicopathological characteristics.
View Article and Find Full Text PDFPurpose: Congenital tracheal stenosis (CTS) is rare and challenging. Complete tracheal rings cause a wide spectrum of airway-obstructing lesions and varying degrees of respiratory distress. Although surgical reconstruction is the primary option for symptomatic CTS, sometimes an appropriate management strategy may be difficult due to other anomalies.
View Article and Find Full Text PDFEur J Obstet Gynecol Reprod Biol
August 2017
A 1-day-old male infant was referred to our department for evaluation of multiple malformations in his oral cavity. He was diagnosed duplication of the pituitary gland-plus syndrome with epignathus, cleft palate, duplication of the mandible, and a lobulated tongue. A thumb-sized mass lesion was visible on the hard palate.
View Article and Find Full Text PDFPurpose: Congenital tracheal stenosis (CTS) is a rare condition and difficult to treat. Slide tracheoplasty has unsatisfactory outcomes for severe neonatal symptomatic CTS. This study evaluated the use of biodegradable polydioxanone stents (BD stent) in a rabbit model of CTS.
View Article and Find Full Text PDFPediatr Surg Int
September 2016
Purpose: The management of esophageal atresia is established, but the rate of postoperative complications remains high. We focused on a new, recently reported method of esophageal elongation using botulinum toxin type A (BTX-A) and evaluated the efficacy of BTX-A injection around esophageal anastomoses with tension in a rabbit model.
Methods: Twenty rabbits aged 8-10 weeks and weighing 1.
Objectives: The safety and efficacy of double-balloon enteroscopy (DBE) in pediatric patients has not been well documented. We aimed to evaluate the clinical efficacy and safety of DBE in children, especially those under 10.
Methods: We retrospectively analyzed our database of DBE procedures performed between September 2000 and September 2013.
Purpose: This study aimed to evaluate the use of a transumbilical incision for infants and children, as well as neonates, with various intraabdominal conditions.
Methods: A retrospective study of transumbilical incision surgery was performed between June 2007 and June 2013. Patients were divided into two groups: group 1 of neonates and group 2 of infants and children.