Serous cystadenoma with fat replacement of the pancreatic body and tail with pancreaticoduodenectomy without pancreatic reconstruction followed by preserved pancreatic endocrine function.

Acquired fat replacement of the pancreatic body and tail is a rare condition that differs from congenital agenesis. A female patient in her 50s presented with a multilocular serous cystic neoplasm located in the pancreatic head extending into the body, accompanied by fat replacement in the pancreatic body and tail. The patient underwent pancreaticoduodenectomy (PD) without remnant pancreatic reconstruction, as the pancreatic duct could not be identified.

Screening of 1-Month-Old Infants With Prolonged QT Interval and Its Cutoff Value.

Background: The prevalence of congenital long QT syndrome (LQTS) (1 : 2,000) is based on genetic testing and ECG data, but the prevalence of electrocardiographically determined prolonged corrected QT interval (pQTc) in infants is unclear.

Methods And Results: Subjects were 10,282 1-month-old infants who participated in 2 prospective ECG screening studies performed in 2010-2011 and 2014-2016. Infants with a QTc ≥0.

A variant in HMMR/HMMR-AS1 is associated with serum alanine aminotransferase levels in the Ryukyu population.

The Ryukyu archipelago is located southwest of the Japanese islands, and people originally from this region, the Ryukyu population, have a unique genetic background distinct from that of other populations, including people from mainland Japan. However, few genetic studies have focused on the Ryukyu population. In this study, we performed genome-wide association studies (GWAS) on the serum levels of alanine aminotransferase (ALT, n = 15,224), aspartate aminotransferase (AST, n = 15,203), and gamma-glutamyl transferase (GGT, n = 14,496) in the Ryukyu population.

Effectiveness of Belimumab for Glucocorticoid Discontinuation in Juvenile-Onset Lupus Nephritis.

Lupus nephritis (LN) is an important complication of systemic lupus erythematosus, for which glucocorticoids (GCs) are the primary treatment. Due to the side effects associated with GCs, their long-term use should ideally be tapered and discontinued. At present, no such possibility exists without problematic flares after discontinuation.

Low Survival Due to Higher Risk of Relapse and Nonrelapse Mortality After Allogeneic HSCT in ATL Compared with AML and ALL.

Background: Patients with adult T-cell leukemia/lymphoma (ATL) are considered to have worse outcomes after allogeneic hematopoietic stem cell transplantation (allo-HSCT) than patients with other hematological malignancies, owing to high risk of relapse and immunocompromised status. However, no studies have compared transplant outcomes between patients with ATL and those with other hematological malignancies using a large-scale database.

Objectives: To compare transplant outcomes between patients with ATL and those with other leukemias and to identify factors contributing to worse transplant outcomes in ATL patients.

A 37-Year Retrospective Analysis Reveals a High Rate of Cutaneous Angiosarcoma of the Scalp in Okinawa, Japan.

Angiosarcoma (AS) is a rare, aggressive malignancy originating from vascular or lymphatic endothelial cells. Despite its severity, little is known about its epidemiology, and no geographical regions have previously been identified as having an exceptionally high incidence. We retrospectively analyzed medical records spanning 37 years (1987-2023) in Okinawa, Japan, identifying 135 cases of AS that were used to calculate its incidence.

Detection of Costal Cartilage Fractures on CT Images With Computer-aided Detection System for Rib Fractures.

Background/aim: Costal cartilage fractures are associated with poor prognosis in patients with blunt chest trauma. A Computer-Aided Detection (CAD) system for detecting rib fractures has been used in practice, but it is unclear whether this system recognizes costal cartilage fractures. This study investigated whether the CAD system for rib fracture can detect costal cartilage fractures.

Adjunctive effects of eltrombopag on immunosuppressive therapy for childhood aplastic anemia.

Eltrombopag is used with first-line immunosuppressive therapy for adult aplastic anemia, although its practical utility in childhood remains unclear. We retrospectively analyzed the outcomes of pediatric patients who received eltrombopag in Japan. Of the 27 eligible patients, 23 (85%) were previously treated, and 15 (56%) had severe or very-severe disease.

A refractory right cervical mediastinal lymphangioma with medication and insertion of a Denver shunt.

The Denver shunt is used for persistent chylothorax as a pleuroperitoneal shunt. Insertion of a Denver shunt was attempted in the current case involving a 3-year-old male with a refractory right cervical mediastinal lymphangioma. He was medicated with Eppikajutsuto® (0.

Sarcomere gene variants did not improve cardiac function in pediatric patients with dilated cardiomyopathy from Japanese cohorts.

Dilated cardiomyopathy (DCM) is a progressive myocardial disorder characterized by impaired cardiac contraction and ventricular dilation. However, some patients with DCM improve when experiencing left ventricular reverse remodeling (LVRR). Currently, the detailed association between genotypes and clinical outcomes, including LVRR, particularly among children, remains uncertain.

Survival benefit of CPAP therapy among dialysis patients with obstructive sleep apnea.

Background And Hypothesis: We observed lower risk of all-cause mortally among continuous positive airway pressure (CPAP) users compared to those non-users using a large polysomnography (PSG) registry. However, the effect of CPAP on mortality risk has not been examined in dialysis patents.

Methods: We studied 9841 patients with PSG performed from September 1990 to 2010 in Nakamura clinic, Okinawa.

A Pediatric Case of Nonprogressive Idiopathic Central Vasculitis: A Spectrum of Clinical Presentations.

In children, the causes of cerebral infarction are varied, and accurate diagnosis and treatment are imperative. An early school-age boy was brought to our hospital due to seizures and impaired consciousness. He was diagnosed with cerebral infarction due to primary central nervous system vasculitis (PCNSV) based on increased inflammatory response and circumferential vessel wall thickening in his right middle cerebral artery.

Clinical Transplant Kidney Function Loss Due to Small Intestinal Bacterial Overgrowth.

Small intestinal bacterial overgrowth (SIBO) is a clinical syndrome involving gastrointestinal symptoms caused by the presence of excessive bacteria in the small intestine. SIBO often leads to diarrhea and poses diagnostic and treatment challenges. Here, we report about a renal transplant recipient who experienced diarrhea-induced hypovolemic shock due to SIBO, necessitating the reintroduction of dialysis, and aim to provide insights to aid health-care providers in diagnosing and managing severe diarrhea in this specific patient group.

A case of falling accident when carrying a thermos bottle in front of the body.

Traumatic injury in children often involves traffic accidents, falls from height, bicycle accidents, abuse, and sports. We present our experience with a 6-year-old girl who bruised her abdomen by falling while running with a thermos bottle hanging diagonally across her neck and left shoulder, crossing in front of her body. After the injury, she was rushed to the hospital and plain abdominal computed tomography revealed free air, suggesting intestinal injury due to blunt trauma.

Optimal timing of bridging annuloplasty and patch augmentation for heterotaxy syndrome associated with functional single ventricles.

Objective: Although the challenging prognosis of functional single ventricles with common atrioventricular valves due to complex morphology and uncontrollable regurgitation by valvuloplasty has been highlighted, reports on when and how these extremely complicated atrioventricular valves should be repaired are few. This study investigated the timing and risk factors for valve intervention in these patients.

Methods: Between April 2006 and March 2023, 40 patients with heterotaxy syndrome associated with functional single ventricles underwent surgery.

Association of schizophrenia with fracture-related femoral neck displacement: A cross-sectional retrospective study.

Aim: Fracture-related femoral neck displacement is more likely in patients with schizophrenia because of delayed diagnosis, as these patients frequently have less severe fracture-associated subjective symptoms. This study aimed to investigate the association of schizophrenia with the risk of fracture-related femoral neck displacement in hospitalized patients.

Methods: We retrospectively analyzed the medical records of patients with femoral neck fractures treated between April 2013 and March 2018 at a single institution.

Infantile Epileptic Spasms Syndrome Complicated by Leigh Syndrome and Leigh-Like Syndrome: A Retrospective, Nationwide, Multicenter Case Series.

Background: Six percent of patients with Leigh syndrome (LS) present with infantile epileptic spasms syndrome (IESS). However, treatment strategies for IESS with LS remain unclear. This retrospective study aimed to evaluate the efficacy and safety of treatment strategies in patients with IESS complicated by LS and Leigh-like syndrome (LLS).

Management of cardiopulmonary bypass in patients with ischemic and hemorrhagic strokes in surgery for active infective endocarditis.

Stroke and intracranial hemorrhage (ICH) are serious complications that are difficult to manage during surgery for active infectious endocarditis (AIE). Relevant society guidelines still recommend delaying the cardiac surgery for AIE with ICH for 4 weeks. Some early studies indicated that the mortality rate decreases when cardiac surgery for ICH is delayed.

Surgical technique for epigastric incisional hernia after omphalocele repair: bilateral modified composite flaps using the upper rectus abdominis muscle and the vertically inverted flap of the lower rectus abdominis fascia.

We present a patient who developed an incisional hernia, from epigastrium to umbilicus, after omphalocele repair. The hernia gradually enlarged to a 10 cm × 10 cm defect with significant rectus abdominis muscle diastasis at the costal arch attachment point. At 6 years of age, the abdominal wall defect in the umbilical region was closed using the components separation technique.

The Japanese Pediatric Continuous Renal Replacement Therapy (jpCRRT) Registry: Study Protocol.

Background: The use of continuous renal replacement therapy (CRRT) in critically ill children is rapidly increasing, but the standard of care has not yet been established and prognosis remains poor. To develop optimal CRRT strategies, we launched a research project generating the Japanese Pediatric CRRT registry, a multicenter registry of CRRT in Japanese pediatric intensive care units (PICUs), to investigate the actual status of CRRT in recent years in PICUs, where data are lacking.

Methods: This manuscript presents a protocol for planning a multicenter prospective registry.

Efficacy of Pharmacotherapy for Seasonal Influenza in Young and Middle-aged Adults: A Systematic Review and Network Meta-analysis.

Objective Seasonal influenza affects healthcare demand. However, the efficacy of anti-influenza drugs, particularly among young patients at a low risk of complications, has rarely been evaluated. Therefore, we evaluated the efficacy of anti-influenza drugs against seasonal influenza in healthy young and middle-aged adults.

Genotype-Phenotype Correlations in 30 Japanese Patients With Congenital Hypothyroidism Attributable to TG Defects.

Context: Thyroglobulin (Tg), encoded by TG, is essential for thyroid hormone synthesis. TG defects result in congenital hypothyroidism (CH). Most reported patients were born before the introduction of newborn screening (NBS).