Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of bone marrow-derived dendritic cells that is uncommon in adults. Its clinical presentation varies depending on the organ involved. Central nervous system (CNS) involvement poses a critical therapeutic challenge because of permanent neurological dysfunction. Here, we report two contrasting cases of adult-onset LCH with distinct clinical outcomes. Case 1 involved a 36-year-old woman presenting with a persistent headache. Neuroimaging revealed a solitary osteolytic lesion in the left parietal bone with dural thickening. Surgical resection was performed, and histopathological examination revealed characteristic Langerhans cells with positive S-100 immunostaining, confirming the diagnosis of LCH limited to the calvarium. The patient was treated with bisphosphonates and remained stable without systemic involvement. Case 2 involved a 26-year-old man presenting with cognitive impairment, somnolence, polyuria, and endocrine dysfunction. Magnetic resonance imaging revealed an enhancing hypothalamic lesion. Histopathological and immunohistochemical analyses confirmed LCH with CD1a and S-100 positivity. BRAF V600E mutation was not detected via pyrosequencing. Systemic chemotherapy with cladribine resulted in partial tumor regression; however, the cognitive dysfunction persisted. These contrasting cases highlight that disease distribution, particularly CNS involvement, influences therapeutic strategies and neurological outcomes in adult LCH. Our findings emphasize that early diagnosis and proactive systemic evaluation are essential as functional recovery remains limited once neurological damage occurs, even with successful cytoreductive therapy.
Primary graft failure is a rare but life-threatening complication after umbilical cord blood transplantation (UCBT), especially in pediatric patients with relapsed acute lymphoblastic leukemia (ALL). Optimal salvage strategies, including conditioning regimens and donor selection for second transplantation, have not been established. This report describes the case of a 9-year-old boy with relapsed B-cell precursor ALL who developed primary graft failure following the first UCBT performed during his second complete remission. The initial transplant had used a cord blood unit with a low CD34-positive cell dose and significant human leukocyte antigen (HLA) and ABO mismatch. Despite myeloablative conditioning, neutrophil engraftment failed, and donor chimerism remained minimal. An urgent second UCBT was performed using a different cord blood unit with improved HLA compatibility and markedly higher total nucleated and CD34-positive cell doses, following reduced-intensity conditioning with fludarabine and low-dose total body irradiation. Sustained neutrophil engraftment was achieved on day +34, with stable full donor chimerism thereafter. Post-transplant complications, including hemophagocytic syndrome, sinusoidal obstruction syndrome, acute graft-versus-host disease, and viral reactivation, were successfully managed. This pediatric case demonstrates that, in the absence of a suitable donor and with prompt intervention, a second UCBT is a feasible salvage option after primary graft failure.
OBJECTIVE:Vaginal cuff recurrence of endometrial cancer is generally managed with radiotherapy, and surgical reports are scarce. We report a laparoscopic resection technique for isolated vaginal cuff recurrence. METHODS:A 57-year-old woman underwent laparoscopic hysterectomy with bilateral salpingo-oophorectomy for grade 1 endometrioid carcinoma (FIGO stage IA) without adjuvant therapy. Nine months postoperatively, she was referred to our hospital with suspected vaginal cuff and pelvic lymph node recurrence. Imaging revealed no invasion of adjacent organs, and lymph nodes were deemed resectable. RESULTS:Laparoscopic resection of the vaginal cuff tumor and pelvic lymphadenectomy were performed. Following the principles of radical hysterectomy, the bilateral cardinal ligaments, uterosacral ligaments, and the anterior and posterior layers of the vesicouterine ligaments were divided. The vaginal canal was circumferentially incised, and the recurrent tumor was excised. As paravaginal tissue invasion was not suspected, the hypogastric nerves were preserved. Specimens were retrieved transvaginally in protective bags. Histopathological examination confirmed recurrent grade 3 endometrioid carcinoma with negative margins and pelvic nodal metastases. Postoperative chemotherapy was administered, and the patient remains disease-free. CONCLUSION:This technique represents a valuable therapeutic option, providing safe and definitive resection for patients with vaginal cuff recurrence.
INTRODUCTION:Mesenteric lymphangioma is a rare cystic lesion that may present as acute abdomen due to events such as axial torsion. This is a case report of a small bowel mesenteric lymphangioma presented with axial torsion, which required emergency surgery after repeated abdominal symptoms and hospital visits failed to identify the lesion. This report includes a review of the literature. CASE PRESENTATION:A 7-year-old boy was presented with abdominal pain and vomiting. Abdominal ultrasound and contrast-enhanced CT revealed the whirlpool sign, leading to a diagnosis of small bowel volvulus. Emergency surgery was performed. A multilocular cyst measuring approximately 8 cm in diameter was identified in the mesentery of the small bowel, approximately 150 cm proximal to the ileocecal junction. The mesentery exhibited volvulus. A partial small bowel resection and cystectomy were performed. Pathological examination revealed irregularly dilated lymphatic lumens with endothelial cells positive for CD31 and D2 - 40, resulting in a diagnosis of mesenteric lymphangioma. CONCLUSIONS:Surgical resection is the standard treatment for mesenteric lymphangioma. Even when symptoms improve with conservative management, this disease carries the potential for serious complications requiring emergency surgery. Therefore, patients presented with abdominal symptoms, this condition should be considered, and early intervention should be contemplated.
OBJECTIVE:Changes in the respiratory function over time in respiratory syncytial virus (RSV)-induced acute bronchiolitis were evaluated by a lung sound analysis. METHODS:We retrospectively examined changes in lung sound parameters over time in 54 children (median age, 7 months) diagnosed with RSV acute bronchiolitis. We evaluated the power of inspiratory and expiratory sounds using a conventional method and analyzed the frequency characteristics of inspiratory sounds using new automated analysis software program. We examined changes in these lung sound parameters over time during and after hospitalization and compared these data with those of age-matched healthy children. RESULTS:In 138 lung sound samples from 54 infants, the power of inspiratory and expiratory sounds was greatest within 4 days after the onset and improved over time. In contrast, the greatest changes in inspiratory sound parameters were observed at 5 to 10 days after the onset of RSV acute bronchiolitis. Some lung sound parameters in patients with RSV acute bronchiolitis did not return to normal even by 14 days after the onset. CONCLUSIONS:This finding is considered a characteristic of RSV acute bronchiolitis in infants and is meaningful in terms of post-treatment management.
OBJECTIVE:Endoscopic retrograde cholangiopancreatography (ERCP) with self-expandable metal stent (SEMS) placement is the standard treatment for malignant biliary obstruction; however, it may not be feasible in cases of duodenal invasion, altered anatomy, or failed cannulation. Although percutaneous transhepatic biliary drainage is a reliable alternative, it is associated with external catheter-related complications and impaired quality of life. Recently, a one-stage percutaneous SEMS placement procedure has been proposed to overcome these limitations. METHODS:We retrospectively reviewed five patients (four women and one man; median age, 68 years) with malignant biliary obstruction who underwent one-stage percutaneous SEMS placement between April 2016 and March 2025 at the Tokai University Hospital. The procedures were performed under ultrasonographic guidance using a 19-gauge EVS™ needle, followed by guidewire exchange, tract dilation with an 8-Fr dilator, and deployment of an Epic™ biliary SEMS. Technical and clinical success and procedure-related complications were evaluated. RESULTS:Herein, the underlying diseases included pancreatic head carcinoma (n = 1), postoperative cholangiocarcinoma with lymph node recurrence (n = 2), postoperative gallbladder carcinoma with lymph node recurrence (n = 1), and postoperative gastric carcinoma with peritoneal dissemination (n = 1). ERCP failure was due to the following reasons: duodenal stenosis, choledochojejunostomy, subtotal stomach-preserving pancreaticoduodenectomy (SSPPD) with cannulation failure, laparoscopic SSPPD not attempted due to inflammation, and Roux-en-Y reconstruction. Three and two patients presented with moderate and mild cholangitis, respectively, as per the Tokyo Guidelines 2018. Technical success was achieved in all patients. No procedure-related complications occurred. CONCLUSION:One-stage percutaneous SEMS placement is a safe and effective option for treating malignant biliary obstruction in patients unsuitable for ERCP or endoscopic ultrasound-guided biliary drainage. With careful case selection, this approach enables rapid biliary decompression without external drainage and may improve the patient's quality of life.
INTRODUCTION:A lumbar hernia, an exceptionally rare abdominal wall defect, accounts for only 1.5-2% of all reported hernia cases. An inguinal hernia is most common type in adults. The simultaneous occurrence of these two distinct hernias is extremely uncommon; only a few cases have been documented to date. Herein, we report a rare case of concomitant iatrogenic lumbar hernia and indirect inguinal hernia that was successfully repaired in a single session using a combined laparoscopic approach. CASE PRESENTATION:A 74-year-old Japanese woman presented with a bulge and pain in the left flank and swelling in the left inguinal region 1 year following lumbar spinal surgery with bone graft harvesting from the left iliac crest. Computed tomography revealed an intestinal herniation above the left iliac crest and an omental herniation in the left inguinal region. Laparoscopic exploration confirmed a 6 cm × 7 cm lumbar defect containing the sigmoid colon and an indirect inguinal hernia. The lumbar hernia was repaired using a Symbotex™ Composite Mesh (20 cm × 15 cm; Medtronic, Minneapolis, MN, USA) fixed with absorbable tacks, and the inguinal hernia was repaired with a Bard® 3D Max® mesh (M size; Davol, Warwick, RI, USA) using the transabdominal preperitoneal (TAPP) approach. The total operative time was 120 min, and the postoperative course was uneventful. The patient was discharged on postoperative day 4. CONCLUSIONS:This case demonstrates the technical feasibility and safety of simultaneous laparoscopic repair of iatrogenic lumbar and inguinal hernias using a combination of intraperitoneal onlay mesh (IPOM) and TAPP techniques. This strategy facilitates minimally invasive, effective, and simultaneous repair under a single anesthesia and hospitalization, highlighting the versatility of laparoscopic surgery in managing complex abdominal wall hernias.
Graft failure remains a major complication in cord blood transplantation (CBT), particularly in patients with nonmalignant diseases, such as inherited metabolic disorders. However, optimal strategies for retransplantation after graft failure remain controversial. Here, we describe a case of mucopolysaccharidosis type II (MPS II) in which durable engraftment was successfully achieved following a second CBT using the same conditioning regimen as the initial transplantation. A 4-year-old boy with MPS II underwent unrelated CBT after undergoing a reduced-intensity conditioning regimen consisting of total abdominal irradiation, fludarabine, and melphalan. He acquired methicillin-resistant Staphylococcus aureus-associated sepsis early in the posttransplant period and developed graft failure on day 28, presumably due to drug-induced myelosuppression rather than insufficient conditioning intensity. Sixty-six days after the first transplantation, a second CBT was performed using the identical conditioning backbone but with antithymocyte globulin as immunosuppressant. The second course was complicated by severe hypercytokinemia with hemophagocytosis, cytomegalovirus antigenemia requiring preemptive antiviral therapy, and upper gastrointestinal bleeding. Nevertheless, donor chimerism remained high, and engraftment was confirmed on day 40 with transfusion independence on day 50. This case highlights the need for an etiological assessment of graft failure and demonstrates the viability of the initial conditioning regimen as a retransplantation strategy when inadequate conditioning is unlikely.
OBJECTIVE:To report a case of dengue fever complicated by acute acalculous cholecystitis (AAC) and hemophagocytic lymphohistiocytosis (HLH), despite initial negative polymerase chain reaction (PCR) results. METHODS:We describe the clinical course of a 40-year-old woman who returned from Indonesia with a persistent fever and rash. The diagnosis was established through clinical evaluation, serological testing, and abdominal imaging. The study conformed to the principles outlined in the Declaration of Helsinki. RESULTS:Although the initial dengue PCR result was negative, subsequent NS1 and IgM antibody test results were positive. The patient developed severe complications, including AAC, characterized by gallbladder wall thickening, and HLH, indicated by marked fever, splenomegaly, hypofibrinogenemia, hyperferritinemia, and high soluble IL-2 receptor levels. Despite these symptoms, the patient was managed conservatively. Planned percutaneous transhepatic gallbladder aspiration was canceled because of spontaneous improvement observed on ultrasonography. The patient recovered fully without corticosteroid or platelet transfusions and was discharged on day 14. CONCLUSION:Given the potential for false-negative PCR results during the acute phase of dengue, multimodal serological testing is essential. This case demonstrates that severe atypical complications, such as AAC and HLH, can be resolved with supportive care alone, avoiding invasive procedures, immunosuppressive therapy, and platelet transfusion.
We present a forensic case of a man in his late 50s who lost consciousness while driving, resulting in a lowspeed (approximately 10 km/h) collision with a wall. He was found unresponsive at the scene and transported to a hospital. Despite resuscitative efforts, he was pronounced dead. A judicial autopsy was conducted 5.5 days postmortem after refrigerated storage. Externally, postmortem marbling was distinctly observed in the head, neck, and upper limbs, whereas the trunk and lower extremities remained unaffected. Internally, approximately 300 mL of hemopericardium was found due to a tear in the anterior wall of the left ventricle. Histological examination revealed acute coronary artery occlusion with intraplaque hemorrhage, indicating an ischemic origin of the myocardial rupture. This case illustrates a rare and regionally selective manifestation of decomposition, with marbling limited to the upper body. We hypothesize that venous congestion caused by cardiac tamponade created a localized microenvironment favorable to bacterial proliferation, facilitating selective marbling. Although refrigeration delays decomposition, it does not prevent it. Our findings demonstrate that antemortem circulatory conditions may influence the regional distribution of early postmortem changes, which should be considered in forensic interpretation.
Drug-induced hypersensitivity syndrome (DIHS), also known as drug reaction with eosinophilia and systemic symptoms, is a severe adverse reaction involving multiple organs. We describe a case of carbamazepine (CBZ)-induced DIHS with rare primary endocrine complications. A 71-year-old Japanese man developed generalized erythroderma with systemic symptoms 6 weeks after the initiation of CBZ at 200 mg/day for trigeminal neuralgia. Clinical evaluation revealed HLA-A*31:01 positivity, marked eosinophilia, elevated inflammatory markers, and multiorgan involvement, including acute interstitial nephritis and primary hypothyroidism. Primary adrenal insufficiency became clinically evident after levothyroxine administration. Chest computed tomography revealed enlarged cervical and axillary lymph nodes, an abscess in the right upper lobe, and emphysema in both lungs. Although HHV-6 reactivation was not detected, the patient's clinical course was consistent with that of atypical DIHS. The patient was treated by immediate discontinuation of CBZ and initiation of systemic corticosteroids after thyroid hormone replacement therapy, which resulted in clinical improvement. To our knowledge, this is the first report of primary adrenal insufficiency as a manifestation of CBZ-induced DIHS in Japan. Carriers of HLA-A*31:01 require comprehensive endocrine and systemic monitoring because DIHS can precipitate rare primary endocrine gland failure. Pre-prescription HLA genotyping remains an essential preventive strategy for this high-risk population.
OBJECTIVE:To establish a practical and reproducible method for procedural standardization during tracheal intubation and ventilatory management during thoracotomy in rats, and evaluate the invasiveness of thoracotomy as a control condition. METHODS:Tracheal length and diameter were measured in adult female Wistar rats to determine appropriate intubation devices. Positive end-expiratory pressure (PEEP) was titrated (0-5 cm H2O) under open chest conditions using a macroscopic lung expansion score. In a separate experiment, the rats underwent left thoracotomy with incision lengths of 1, 2, or 3 cm, followed by macroscopic and histological assessments 4 weeks later. RESULTS:Mean tracheal length and inner diameter were 59.7 and 1.63 mm, respectively. A 16-gauge, 48-mm intravenous catheter was suitable for intubation. Optimal lung expansion was achieved at a PEEP of 4 cm H2O. Macroscopic pleural adhesions and post-operative weight loss were not observed in any thoracotomy groups. Histological examination revealed mild pleural thickening and fibrin deposition without differences in incision length. CONCLUSIONS:A standardized intubation and ventilatory protocol with PEEP set at 4 cm H2O provides stable lung expansion during thoracotomy in rats. A thoracotomy up to 3 cm in length induces minimal pathological changes and is suitable as a control condition in experimental thoracic surgery models.
OBJECTIVE:Japanese Traditional (Kampo) Medicine (JTM) relies on traditional diagnostic patterns (sho). However, half of Japanese physicians do not consider Kampo patterns when prescribing, increasing the risk of improper use and adverse events. This study evaluated the accuracy and challenges of KAMPO365, a diagnostic support system for JTM, by comparing its outputs with diagnoses and prescriptions by certified physicians of the Japan Society for Oriental Medicine. METHODS:Twenty outpatients at two hospitals participated by inputting clinical data into KAMPO365, which generated Kampo pattern diagnoses and formula recommendations. Specialists, blinded to KAMPO365 outputs, conducted independent diagnoses and prescribed five candidate Kampo formulas. RESULTS:The results showed a 35% concordance rate between KAMPO365-recommended formulas and specialists' selections, with higher agreement in pharmacological classification (45%). Concordance rates for diagnostic patterns were 55% for deficiency/excess and 65% for cold/heat. For qi, blood, and fluid disturbance patterns, fluid retention had the highest concordance (75%), while qi counter flow and blood deficiency had the lowest (30%). CONCLUSION:KAMPO365 demonstrated potential in aiding non-specialists, though improvements in diagnostic algorithms and the coverage of Kampo formulas are needed. Despite its limitations, this study suggests the potential utility of KAMPO365 for diagnosing Kampo patterns and providing precise prescriptions.
OBJECTIVE:To describe a simple and reproducible rat pneumothorax model that is suitable for future sealant testing. METHODS:Female Wistar rats (16 weeks) underwent left thoracotomy under mechanical ventilation. While the lung was inflated, the visceral pleural surface was cauterized using monopolar electrocautery with a ball electrode (coagulation mode, power 20%, 0.5 s), and then punctured with a 21G needle to a depth of 0.5 cm (Model E). The air-leak threshold airway pressure was measured using a water seal test. Pneumothorax and re-expansion were assessed by fluoroscopy, and pleural repair was evaluated histologically at 4 weeks. RESULTS:Pneumothorax was induced in all rats (12/12) without procedure-related mortality. The air-leak threshold pressure ranged from 12 to 25 cm H2O (mean 17.3; median 16). All rats showed complete lung re-expansion at 1 week. Histological findings revealed fibrotic pleural repair (~100 nm thick) with preserved deep alveolar architecture. CONCLUSION:Electrocautery-assisted pleural puncture provides a feasible and reproducible rat pneumothorax model for subsequent preclinical studies.
A 74-year-old man presented with a persistent cough, peripheral blood leukocytosis, and lung opacities. No lesions were identified in the upper airways or kidneys, and the anti-neutrophil cytoplasmic antibodies (ANCA) were negative. The initial clinical presentation was relatively mild and compatible with post-infectious organizing pneumonia; however, the presence of multinucleated giant cells, microabscesses, small granuloma-like lesions, and focal fragmentation/disruption of the vascular elastic laminae, in conjunction with organizing pneumonia in the lung parenchyma, were suggestive of granulomatosis with polyangiitis (GPA). A multi-disciplinary diagnosis, integrating clinical, radiological, and pathological findings, is useful for the early diagnosis of ANCA-negative, lung-limited GPA, which may later recur and require intensive immunosuppressive therapy.
The aim of this study was to clarify the effects of reduced frame rates in videofluoroscopic swallowing studies (VFSS) on aspiration assessment. The consecutive key frames (CKFs), that is, the range of key frames that are essential for penetration-aspiration scale (PAS) scoring of abnormal laryngeal penetration (ALP) and aspiration, was defined using retrospective 30-frames/second (fps) VFSS records for 50 adult dysphagia patients at an acute care university hospital. The results showed that the CKFs was larger in 24 aspiration patients than in 26 ALP patients, and its distribution was wider. The minimum CKFs that included no trace of residual liquid (TRL) was 8 for aspiration. When reducing the frame rate in seven steps to 3.75 fps, the complete disappearance of CKFs with no TRL was seen in only four ALP patients. With partial disappearance of CKFs, the PAS scoring of ALP and aspiration even with a reduction to 3.75 fps agreed consistently and almost completely with that at 30 fps, and high reliability was obtained. This study was exploratory, but 3.75 fps was the lower limit that satisfied the criteria in analyses after defining the acceptable level for frame rate reductions in assessing aspiration.
OBJECTIVE:Childhood asthma can lead to chronic obstructive pulmonary disease in adults, thus necessitating an early diagnosis and timely intervention from early childhood. METHODS:A retrospective study was conducted on parameters related to lung sound power in three-year-old healthy children. All cases were surveyed using the ATS-DLD questionnaire, which mainly covered items related to the history of wheezing, diagnosis of asthma/asthmatic bronchitis, and history of allergies, and then lung sounds were measured. From these data, the characteristics of inspiratory and expiratory sound power at this age were examined. RESULTS:There were correlations between the parameters related to lung sound power at the low- and middle-range frequencies. In addition, children with a history of wheezing or diagnosed with asthma/asthmatic bronchitis showed an increase in the power of inspiratory and expiratory sounds and the power ratio of inspiratory and expiratory sounds compared with children without such histories. Furthermore, the parameters of lung sound power correlated with those of the inspiratory sound spectrum. CONCLUSIONS:Measurements of the power of inspiratory and expiratory sounds confirmed that three-year-old children with a history of wheezing or diagnosed asthma/asthmatic bronchitis had characteristic lung sounds even when healthy.
A 72-year-old man presented with a four-month history of muscle weakness and pain. Serum creatine kinase level was markedly elevated at 22,720 IU/mL, and magnetic resonance imaging revealed high signal intensity in the thigh and upper arm muscles. The presence of an autoantibody against 3-hydroxy-3-methylglutaryl-coA reductase (HMGCR) in the serum confirmed the diagnosis of immune-mediated necrotizing myositis. Subsequent systemic examination revealed primary lung carcinoma in the right lower lobe. Despite the initial corticosteroid therapy, there was no improvement in muscle symptoms. Surgical resection of the tumor, followed by additional intravenous immunoglobulin therapy, controlled necrotizing myositis. The postoperative pathological diagnosis was pleomorphic carcinoma. Despite adjuvant chemotherapy, lung cancer recurred within six months, leading to the patient's death. This case suggests that while treatment of malignancy can be effective for tumor-associated syndromes, such as anti-HMGCR antibody-positive necrotizing myositis, immunosuppressive therapy used for myositis may be a ssociated with an increased r isk of cancer recurrence.
PURPOSE:The purpose of this study was to obtain basic data for stress management by clarifying the current state of stress among the general public and how they cope with stress. METHOD:An online survey was conducted on 100,000 men and women registered with an online survey company. RESULTS:Respondents with stress test scores of 77 or more were classified as the high-stress group and those with scores below 77 were classified as the non-high-stress group. The proportion of men and women in the high-stress group was 14.8% and 17.7%, respectively. By age, the proportion of men and women in their 20s and 30s was significantly higher than other age groups. For both men and women, the most common ways to deal with stress were "sleeping" and "bathing." In the high-stress group, the most common ways to deal with stress were "receiving chiropractic massage" for men and "spending time in the bathroom" for women. A binomial logistic regression analysis revealed that factors influencing the high-stress group included "short sleep time," "long working hours," and "low household income." CONCLUSIONS:The stress factors and coping methods revealed in this study are considered to be important health issues for the entire nation, and we believe that this report will contribute to preventive medicine and public health, which aim to reduce stress among the general public.
OBJECTIVE:In Japan, many individuals with mental disorders require long-term hospitalization. However, to promote community integration and enable these individuals to live comfortably in their local areas, mental and physical care provided by nurses is essential. Therefore, this study aimed to examine the status of home-visiting health professionals and determine the challenges in providing support to patients with physical and mental health problems. METHODS:Focus group interviews with 30 professionals from eight home-visit nursing stations were conducted and analyzed using thematic analysis. RESULTS:The difficulties in providing physical care to patients with mental illness were categorized into three themes: (1) "fragmentation of healthcare, isolation, and adverse effects of long-term retention," arising from non-integrated medical care for individuals with mental disorders; (2) "background of patients that makes it difficult to participate in physical healthcare," stemming from mental symptoms or the patient's own perception; and (3) "loneliness of the patients concerned and lack of security in medical care". CONCLUSION:The results showed that Japanese home-visiting health professionals faced several challenges in providing physical care to individuals with mental disorders. To improve patient care, this study suggests that societal efforts addressing the identified challenges are necessary.
