Fukushima Journal of Medical Science最新文献

Aim: To clarify whether the response to treatment of IgA nephropathy (IgAN) differs depending on patient age, we examined the response to treatment according to age of onset in children with IgAN.

Methods: We collected data for 44 children with severe IgAN. The children were retrospectively divided into three groups based on their age at disease onset. Group 1 consisted of 24 children under 11 years old, group 2 consisted of 9 children aged 12 to 13 years, and group 3 consisted of 11 children aged over 14 years old. The clinical features and prognosis were analyzed for each group.

Results: The urinary protein excretion and serum IgA values in group 3 were higher than those in groups 1 and 2 at the most recent follow up, and histological findings showed that the MESTCG scores in group 3 were higher than those in group 1. Furthermore, the incidence of patients with persistent nephropathy or renal insufficiency in group 3 was higher than those in groups 1 and 2.

Conclusions: Patients aged 14 years and older with IgAN may respond poorly to treatment compared with those younger than 14 years old. Therefore, care must be taken regarding response to treatment and relapse when treating older children.

Neurofibromatosis type 2 (NF2), a multiple neoplasia syndrome, is a manifestation of an impaired expression of the merlin protein, exerting inhibitory effects on cell proliferation signals due to abnormalities of the NF2 gene located on chromosome 22. About half of patients inherit a germline mutation from a parent, and nearly 60% of de novo NF2 patients are estimated to have somatic mosaicism. The development of technical methods to detect NF2 gene mutation, including targeted deep sequencing from multiple tissues, improved the diagnostic rate of mosaic NF2. With improved understanding of genetics and pathogenesis, the diagnostic criteria for NF2 were updated to assist in identifying and diagnosing NF2 at an earlier stage. The understanding of cell signaling pathways interacting with merlin has led to the development of molecular-targeted therapies. Currently, several translational studies are searching for possible therapeutic agents targeting VEGF or VEGF receptors. Bevacizumab, an anti-VEGF monoclonal antibody, is widely used in many clinical trials aiming for hearing improvement or tumor volume control. Currently, a randomized, double-masked trial to assess bevacizumab is underway. In this randomized control trial, 12 other Japanese institutions joined the principal investigators in the clinical trial originating at Fukushima Medical University. In this review, we will be discussing the latest research developments regarding NF2 pathophysiology, including molecular biology, diagnosis, and novel therapeutics.

Alzheimer's disease (AD) is diagnosed by the presence of both amyloid β and tau proteins. Recent advances in molecular PET imaging have made it possible to assess the accumulation of these proteins in the living brain. PET ligands have been developed that bind to 3R/4R tau in AD, but not to 3R tau or 4R tau alone. Of the first-generation PET ligands, ¹⁸F-flortaucipir has recently been approved by the Food and Drug Administration. Several second-generation PET probes with less off-target binding have been developed and are being applied clinically. Visual interpretation of tau PET should be based on neuropathological neurofibrillary tangle staging instead of a simple positive or negative classification. Four visual read classifications have been proposed: "no uptake," "medial temporal lobe (MTL) only," "MTL AND," and "outside MTL." As an adjunct to visual interpretation, quantitative analysis has been proposed using MRI-based native space FreeSurfer parcellations. The standardized uptake value ratio of the target area is measured using the cerebellar gray matter as a reference region. In the near future, the Centiloid scale of tau PET is expected to be used as a harmonized value for standardizing each analytical method or PET ligand used, similar to amyloid PET.

Accurate and efficient self-assessment is a critical skill for medical students to develop as part of their professional development. Along with clinical training reform at Fukushima Medical University, rubric-based student self-assessment and teacher assessment of students' performance using our proposed assessment tool, which includes several aspects of clinical skills and abilities, was initiated to improve the clinical clerkship process. To investigate how students identified their weaknesses and strengths, we analyzed the results of 119 fourth-year medical students' self-assessment and corresponding teacher assessment. Our study revealed substantial consistency between student self-assessment and teacher assessment, despite some overestimation and underestimation in student self-assessments. Students who incorrectly assess themselves require a variety of feedback to increase their self-efficacy and self-confidence, as well as to identify their weaknesses.

We describe four cases of pseudomyxoma peritonei (PMP) that were diagnosed and treated at our hospital.Case 1: A 26-year-old woman with a large multicystic ovarian tumor and massive ascites was diagnosed with PMP originating from a borderline mucinous ovarian tumor. She underwent fertility-preserving staging laparotomy and was treated with three courses of intraperitoneal chemotherapy. There has been no recurrence in the 15 years since her first operation. Case 2: A 72-year-old woman with a giant ovarian tumor and massive ascites was diagnosed with PMP originating from low-grade appendiceal mucinous neoplasm (LAMN). After laparotomy, the patient was managed conservatively because she did not want aggressive treatment. She has remained asymptomatic with a small amount of ascites for 3 years. Case 3: A 82-year-old woman with ovarian tumors, massive ascites, and suspected PMP underwent emergency laparotomy due to appendiceal perforation and pan-peritonitis. She was diagnosed with PMP originating from LAMN. She has remained asymptomatic with a small amount of ascites for 2 years. Case 4: A 42-year-old woman with multicystic ovarian tumors and massive ascites underwent laparotomy. She was diagnosed with PMP originating from LAMN. Since multidisciplinary treatment was indicated and desired, the patient was referred to a specialized facility where cytoreductive surgery and hyperthermic intraperitoneal chemotherapy was performed. The patient has done well since the treatment.Although most cases of PMP originate from mucinous tumors of the appendix, female patients with PMP often present with ovarian tumors and are commonly referred to gynecology clinics. It is therefore important for gynecologists to be familiar with PMP and to be able to diagnose it accurately and select the most suitable management including multidisciplinary treatments.

Objectives: Methotrexate (MTX) is associated with extensive side effects, including myelosuppression, interstitial pneumonia, and infection. It is, therefore, critical to establish whether its administration is required after achieving remission with tocilizumab (TCZ) and MTX combination therapy in patients with rheumatoid arthritis (RA). Therefore, the aim of this multicenter, observational, cohort study was to evaluate the feasibility of MTX discontinuation for the safety of these patients.

Methods: Patients with RA were administered TCZ, with or without MTX, for 3 years; those who received TCZ+MTX combination therapy were selected. After remission was achieved, MTX was discontinued without flare development in one group (discontinued [DISC] group, n = 33) and continued without flare development in another group (maintain [MAIN] group, n = 37). The clinical efficacy of TCZ+MTX therapy, patient background characteristics, and adverse events were compared between groups.

Results: The disease activity score in 28 joints-erythrocyte sedimentation rate (DAS28-ESR) at 3, 6, and 9 months was significantly lower in the DISC group (P < .05, P < .01, and P < .01, respectively). Further, the DAS28-ESR remission rate at 6 and 9 months and Boolean remission rate at 6 months were significantly higher in the DISC group (P < .01 for all). Disease duration was significantly longer in the DISC group (P < .05). Furthermore, the number of patients with stage 4 RA was significantly higher in the DISC group (P < .01).

Conclusions: Once remission was achieved, MTX was discontinued in patients who responded favorably to TCZ+MTX therapy, despite the prolonged disease duration and stage progression.

Cardiac angiosarcoma is a rare malignant tumor with a poor prognosis, characterized by the high uptake of ¹⁸F-fluorodeoxyglucose (FDG). This case report presents two cases of cardiac angiosarcoma with a marked difference in FDG uptake and prognosis.Case Summary:Case 1: A 40-year-old male presented with syncope. Ultrasound echocardiography demonstrated a cardiac tumor with a high uptake of ¹⁸F-FDG (maximum standardized uptake value=9.2). The patient underwent heart catheterization and tumor biopsy. The pathological result was high-grade angiosarcoma, and the MIB-1(Ki-67) proliferation index was approximately 20%. Systemic chemotherapy was administered; however, the patient died 2 years and 5 months after disease onset.Case 2: A 65-year-old female had a right atrial tumor incidentally diagnosed during routine ultrasound echocardiography. The tumor exhibited a low uptake of ¹⁸F-FDG (maximum standardized uptake value=1.8). Open heart surgery was performed, and the tumor was completely resected. Histological analysis revealed low-grade angiosarcoma, and the MIB-1(Ki-67) proliferation index was less than 5%. The patient was followed-up and had not relapsed 2 years after surgery.Conclusion: ¹⁸F-FDG uptake may reflect pathological tumor grade and prognosis in cardiac angiosarcoma.

We encountered a very rare case of fulminant necrotizing enterocolitis (F-NEC) in a preterm male baby. The course of NEC and sepsis in this case was clearly different from the usual course. After onset at 14 days of life, catheter-related bloodstream infection was first assumed, and antibiotics and γ-globulin administration were started. However, 12 hours after onset, the baby's abdominal distension increased remarkably, and his entire abdominal wall turned red to purple. Escherichia coli were isolated from the blood culture, but the catheter tip culture was negative. Exchange transfusion was performed 32 hours after onset, but no significant changes were observed in the baby's general condition, and he died 46 hours after onset. The acute phase reactants of CRP and α1-acid glycoprotein increased, but haptoglobin did not. Although IL-1β and TNFα increased as expected with sepsis, IL-6, IL-8, IL-10, and G-CSF however increased to a greater extent than expected. From the above, we diagnosed the development of intestinal necrosis as a result of widespread intestinal ischemia, and that sepsis was associated with this poor condition.

Background: Carcinoid tumors of the ovary are rare tumors, histopathologically classified as monodermal teratomas and somatic-type tumors arising from dermoid cysts. Their malignancy varies from borderline to malignant. Carcinoid tumors can occur in young and elderly women, and are sometimes seen in mature teratoma, struma ovarii, or mucinous cystadenoma as a nodule or tumor. Strumal carcinoid and mucinous carcinoid present as special types of carcinoid tumors of the ovary.

Case report: This report describes a 56-year-old woman who presented with a large pelvic mass on abdominal ultrasonography during a medical examination. The diameter of the pelvic tumor was approximately 11 cm and was suspected to be ovarian cancer. The values of CA125 and CEA were above their reference intervals on preoperative examination. Abdominal total hysterectomy and bilateral salpingo-oophorectomy were performed. Intraoperative frozen-section histopathology suggested a diagnosis of mucinous adenocarcinoma; therefore, partial omentectomy and pelvic lymphadenectomy were also performed. Permanent-section histopathology led to a final diagnosis of strumal carcinoid of the ovary, stage IA (FIGO 2014). Six years post-operation, the patient had no sign of recurrence.

Lower back pain (LBP) is common among baseball players, and the occurrence of lumbar intervertebral disc degeneration is high. The dynamic load on the lumbar spine due to the postures and movements characteristic of baseball is suspected of aggravating LBP caused by degeneration, but the difference in batting action between players with and without degeneration is not known. The purpose of this study was to investigate the difference in batting motion in the presence and absence of lumbar disc degeneration (LDD). The subjects were 18 male baseball players belonging to the University League Division I: seven with disc degeneration and 11 without. The motion task analyzed tee batting. The items examined were the angles of rotation of shoulder, pelvis, hip, and twisting motion; rotation angular velocity; time to maximum angular velocity; and muscle activity potentials of the bilateral latissimus dorsi, erector spinae, multifidus, external oblique, internal oblique, rectus abdominis, and gluteus medius muscles; at each stage of batting action. There were significant differences between the shoulder and pelvis in rotation angle, time to maximum angular velocity, and muscle activity in the presence and absence of LDD, and in the time to maximum angular velocity between the shoulder and pelvis. We infer that these differences are characteristic of batting motion due to LDD.