Mucocuaneous Lymph Node Syndrome(Kawasaki disease) is a new diseae entity that was first described by Kawasakiin 1967. It occures predominantly in children less than 5yrs old and acute febrile illness, which is mucocutaneousinvolvment associated with swelling of cervical lymph nodes. The coronary artery aneurysms have been revealed 20–30% of patients with Kawsaki disease. The authors report a case of multiple coronary artery aneurysms inKawasaki disease which was diagnosed by a coronary arteriography.
Aneurysm ; Angiography ; Child ; Coronary Vessels ; Humans ; Lymph Nodes ; Mucocutaneous Lymph Node Syndrome

PURPOSE: Longitudinal pancreaticojejunostomy is a treatment option for selected patients with chronic obstructive pancreatitis that is rarely performed laparoscopically. METHODS: A 54-year-old man presented with chronic postprandial abdominal pain. A computed tomography scan revealed multiple calcified stones at the pancreatic head and tail with marked dilation of the pancreatic duct (0.8 cm in diameter). The patient underwent laparoscopic longitudinal pancreticojejunostomy. Impacted stones were removed and Roux-en-Y pancreaticojejunostomy was performed using an intracorporeal suture technique in a longitudinal side-to-side manner. RESULTS: The total operation time and estimated blood loss were 150 min and 50 ml, respectively. The patient tolerated a regular diet without postprandial abdominal pain. Postoperative recovery was uneventful and the patient was discharged on postoperative day 5 in good condition. He returned to work on postoperative day 9. CONCLUSION: Longitudinal pancreaticojejunostomy might be an effective option for relief of chronic pain in treating chronic obstructive pancreatitis.
Abdominal Pain ; Chronic Pain ; Diet ; Head ; Humans ; Middle Aged ; Minimally Invasive Surgical Procedures ; Pancreatic Ducts ; Pancreaticojejunostomy* ; Pancreatitis* ; Pancreatitis, Chronic ; Suture Techniques ; Tail

Adult ; Hand ; Humans ; Parietal Bone ; Rabeprazole ; Skull ; Sutures ; Tissue Donors ; Transplants

The recent advances in human pluripotent stem cells (hPSCs) enable to precisely edit the desired bases in hPSCs to be used for the establishment of isogenic disease models and autologous ex vivo cell therapy. The knock-in approach based on the homologous directed repair with Cas9 endonuclease, causing DNA double-strand breaks (DSBs), produces not only insertion and deletion (indel) mutations but also deleterious large deletions. On the contrary, due to the lack of Cas9 endonuclease activity, base editors (BEs) such as adenine base editor (ABE) and cytosine base editor (CBE) allow precise base substitution by conjugated deaminase activity, free from DSB formation. Despite the limitation of BEs in transition substitution, precise base editing by BEs with no massive off-targets is suggested to be a prospective alternative in hPSCs for clinical applications. Considering the unique cellular characteristics of hPSCs, a few points should be considered. Herein, we describe an updated and optimized protocol for base editing in hPSCs. We also describe an improved methodology for CBE-based C to T substitutions, which are generally lower than A to G substitutions in hPSCs.

The sequential change from totipotency to multipotency occurs during early mammalian embryo development. However, due to the lack of cellular models to recapitulate the distinct potency of stem cells at each stage, their molecular and cellular characteristics remain ambiguous. The establishment of isogenic naïve and primed pluripotent stem cells to represent the pluripotency in the inner cell mass of the pre-implantation blastocyst and in the epiblast from the post-implantation embryo allows the understanding of the distinctive characteristics of two different states of pluripotent stem cells. This review discusses the prominent disparities between naïve and primed pluripotency, including signaling pathways, metabolism, and epigenetic status, ultimately facilitating a comprehensive understanding of their significance during early mammalian embryonic development.

The sequential change from totipotency to multipotency occurs during early mammalian embryo development. However, due to the lack of cellular models to recapitulate the distinct potency of stem cells at each stage, their molecular and cellular characteristics remain ambiguous. The establishment of isogenic naïve and primed pluripotent stem cells to represent the pluripotency in the inner cell mass of the pre-implantation blastocyst and in the epiblast from the post-implantation embryo allows the understanding of the distinctive characteristics of two different states of pluripotent stem cells. This review discusses the prominent disparities between naïve and primed pluripotency, including signaling pathways, metabolism, and epigenetic status, ultimately facilitating a comprehensive understanding of their significance during early mammalian embryonic development.

The sequential change from totipotency to multipotency occurs during early mammalian embryo development. However, due to the lack of cellular models to recapitulate the distinct potency of stem cells at each stage, their molecular and cellular characteristics remain ambiguous. The establishment of isogenic naïve and primed pluripotent stem cells to represent the pluripotency in the inner cell mass of the pre-implantation blastocyst and in the epiblast from the post-implantation embryo allows the understanding of the distinctive characteristics of two different states of pluripotent stem cells. This review discusses the prominent disparities between naïve and primed pluripotency, including signaling pathways, metabolism, and epigenetic status, ultimately facilitating a comprehensive understanding of their significance during early mammalian embryonic development.

In restorative treatment using fixed dental prostheses, dentists should select appropriate restoration material among various types of dental materials. The strength, marginal fit, esthetics, wear resistance, biocompatibility, and cost are important factors in the choice of restoration materials. The present case showed a surface stain on a monolithic zirconia restoration that was due to wear between the monolithic zirconia restoration and the base metal alloy restoration. This phenomenon was confirmed by surface roughness measurement and electron probe micro-analysis.
Alloys ; Dental Alloys ; Dental Materials ; Dental Prosthesis ; Dentists ; Esthetics ; Humans

Hepatic glycogenosis is an under-recognized cause of hepatomegaly and elevated serum aminotransferase levels in patients with type 1 diabetes; further, most cases of hepatic glycogenosis are reported to be associated with poor glycemic control. In this report, we describe the case of a 12-year-old girl with hepatic glycogenosis, who presented with elevated liver enzymes. She was diagnosed with type 1 diabetes at the age of 6 years, and her diabetes was very "brittle" with recurrent episodes of hypoglycemic attacks. Her recent hemoglobin A1C (HbA1c) level was 8.4%, and the average HbA1c level during the last 2 years was 8.7%. Her aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were persistently elevated during the last year, up to 700 and 258 U/L, respectively. Her growth rate and pubertal development were normal. Her serum was negative for hepatitis viral markers, and the plasma levels of ceruloplasmin and ferritin were also normal. Ultrasound examination revealed hepatomegaly with increased hepatic echogenicity. Liver biopsy demonstrated irregular glycogen deposition in hepatocytes. Recurrent hypoglycemia was resolved with continuous subcutaneous insulin infusion, and after 3 weeks, her AST and ALT levels decreased to 47 and 33 U/L, respectively. We conclude that hepatic glycogenosis should be suspected as a cause of abnormal liver function tests in patients with poorly controlled or brittle type 1 diabetes. After excluding other causes of hepatic dysfunction, a 1-month trial for achieving improved glycemic control, while avoiding hypoglycemia, is recommended before proceeding with invasive investigation of the patient.
Alanine Transaminase ; Aspartate Aminotransferases ; Biomarkers ; Biopsy ; Ceruloplasmin ; Child ; Diabetes Mellitus ; Ferritins ; Glycogen ; Glycogen Storage Disease ; Hemoglobins ; Hepatitis ; Hepatocytes ; Hepatomegaly ; Humans ; Hypoglycemia ; Insulin ; Liver ; Liver Diseases ; Liver Function Tests ; Liver Glycogen ; Plasma

Undifferentiated(embryonal) sarcoma of the liver developing predominantly in the late childhood is extremely rare in malignant hepatic tumors. It has been described by a variety of terms such as malignant mesenchymoma, embryonal sarcoma, fibromyxosarcoma, embryonal rhabdomyosarcoma or primary sarcoma of the liver. The tumor usually presents a huge mass containing solid and sometimes cystic components, and is cmposed of large stellate and spindle cells in an abundant myxoid matrix. The treatment of choice is surgical resection with or without adjuvant chemotherapy and/or radiotherapy, but the prognosis of this disease has been reported extremly poor. We experienced a case of undifferentiated(embryonal) sarcoma of the liver recently which was confirmed by pathological examinations in a 18-year-old boy. He visited our hospital for examination of the progressive growing RUQ abdominal mass detected incidentally. On physical examination, a firm, non-tender, hard, fixed, huge, abdominal mass was palpated in the right upper quadrant of abdomen. Abdominal ultrasonography, computerized tomography and magnetic resonance imaging showed a 10x15cm sized mass confined to the left lobe of the liver, and it was hypovascular mass on angiography. We performed left lobectomy including the tumor and it was confirmed as undifferentiated (embryonal) sarcoma of the liver by pathologic examination. We herein report this case including histologic, electronmicroscopic and flow cytometric results with the review of literatures.
Abdomen ; Adolescent ; Angiography ; Chemotherapy, Adjuvant ; Humans ; Liver* ; Magnetic Resonance Imaging ; Male ; Mesenchymoma ; Physical Examination ; Prognosis ; Radiotherapy ; Rhabdomyosarcoma, Embryonal ; Sarcoma* ; Ultrasonography