Multiple myeloma (MM) is an incurable hematological malignancy. Introduction of autologous stem cell transplantation, as well as proteasome inhibitors and immunomodulatory drugs (IMiDs) has improved the treatment results. However, many patients develop resistance to existing therapies, and novel treatment strategies for these patients must be established. Therapeutic antibodies including daratumumab or isatuximab targeting CD38 and elotuzumab targeting SLAMF7 have been introduced in the clinic as immunotherapies for MM. These antibodies exert cytotoxic effects on myeloma cells through the activation of effector cells such as natural killer cells and induction of phagocytosis by macrophages. The recent studies suggest the possibility of therapeutic antibodies to bring about influence on testing in treatment of MM. CD38 is one of the antigens used for detection of myeloma cells in flowcytometry. The use of daratumumab is supposed to downregulate CD38 expression in myeloma cells. Thus, daratumumab treatment may affect the evaluation of minimal residual disease (MRD) by flow cytometry. Immunofixation electrophoresis (IFE) to detect monoclonal protein is essential for evaluation of response to treatment. Both daratumumab and elotuzumab are derived from human IgG-k and their administration may affect the evaluation of immunofixation electrophoresis if the tumor-derived M protein belongs to the same IgG-k subclass. To overcome the influence of therapeutic antibodies on testing, a method for detecting MRD using different antibodies from conventional ones and daratumumab-specific immunofixation electrophoresis reflex assay (DIRA) for IFE have been introduced. In this review, progress of therapeutic antibodies and possible impacts of these antibodies on the evaluation of response to the treatment will be discussed.

Objectives: To examine the predictors of prolonged dysphagia at discharge in cardiovascular patients treated with invasive and non-invasive mechanical ventilation (MV).

Methods: Inpatients with dysphagia and cardiovascular disease treated with MV at Japanese Red Cross Ashikaga Hospital were evaluated between April 2019 and August 2022 in this retrospective observational study. Age, sex, ambulatory ability, Geriatric Nutritional Risk Index (GNRI), primary heart disease, Charlson Comorbidity Index, invasive or non-invasive MV, Sequential Organ Failure Assessment, duration of MV, days from the withdrawal of MV to the start of rehabilitation, Functional Oral Intake Scale (FOIS) at the start of rehabilitation and at discharge, length of hospital stay, training time per day, pneumonia after the start of rehabilitation, and death during hospitalization were surveyed. To assess the predictors of death and dysphagia at discharge, univariate comparisons and Firth's logistic regression analyses were performed.

Results: Ten of the 128 patients died, and 71 patients had dysphagia at discharge. Lower FOIS scores (P = 0.04), less training time per day (P < 0.01), and pneumonia (P = 0.02) were significantly associated with death in univariate comparisons. Ambulatory ability (odds ratio = 0.09; 95% confidence interval = 0.01-1.48, P = 0.04) and the nutritional risk assessed by GNRI (odds ratio = 1.84; 95% confidence interval = 1.20-2.81, P < 0.01) were the independent predictors of dysphagia at discharge in Firth's logistic regression analysis.

Conclusions: Ambulatory ability and GNRI are valuable as the predictors of prolonged dysphagia at discharge in cardiovascular patients treated with MV.

Prader-Willi syndrome is a rare neurodevelopmental disorder that is characterized by neonatal hypotonia, hypogonadism, hyperphagia, and cognitive impairment. We compared the quality of life of caregivers of patients with Prader-Willi syndrome before and after the patients received growth hormone therapy.

Caregivers' quality of life was assessed using the Japanese version of the World Health Organization Quality of Life 26 (WHO-QOL26). We examined the impacts of two genotypes of Prader-Willi syndrome (deletion and maternal uniparental disomy) and growth hormone therapy on caregivers' quality of life. Using paired t-tests, we compared the caregivers' quality of life scores before and after the patients with Prader-Willi syndrome received growth hormone therapy.

The study included 141 individuals with Prader-Willi syndrome (68 males and 73 females) definitively identified with the deletion genotype and 35 (13 males and 22 females) who exhibited maternal uniparental disomy on chromosome 15. Our findings revealed that the caregivers' quality of life scores of the individuals with Prader-Willi syndrome-deletion before and after growth hormone therapy differed significantly. On the contrary, a statistically significant difference was not noted in all domains of quality of life scores of the caregivers of patients with Prader-Willi syndrome-maternal uniparental disomy on chromosome 15 genotype before and after growth hormone therapy.

The results of this study highlight that the caregivers of individuals with Prader Willi syndrome, particularly those with the Prader-Willi syndrome-deletion genotype receiving growth hormone therapy, had improved quality of life across multiple domains, including physical health, psychological well-being, social connections, and environmental experience.

Background: Chronic Myeloid Leukemia (CML) is a hematological malignancy caused by an abnormality of the Philadelphia chromosome. Along with the development of CML treatment, the discovery of tyrosine kinase inhibitors (TKI) paved the way for CML patient and lead to longer survival rate. It is very important to know whether the treatment response is successful or not as well as the predictive factors that can improve the therapeutic response of CML patients treated with TKI.

Methods: This study is an observational cohort study conducted for 12 months long and involving 40 CML patients treated with TKI, both hematological and molecular therapy response were evaluated in this study. We analyzed some predictive factors such as relationship between early clinical and laboratory symptoms before the treatment by TKI and treatment response and the relationship between Eutos, Sokal and Hasford scores and treatment response.

Results: A total of 40 chronic phase CML patients, consist of 25 men (62.5%) and 15 women (37.5%) with mean age of 37 years. Most common clinical manifestation was weight loss (100%), followed by splenomegaly in 39 people (97.5%). Complete Hematologic Response (CHR) after 3 months of TKI therapy was achieved by 15 patients (37.5%). The number of patients who achieved 6-months CHR increased to 19 patients (47.5%). Among 37 samples who performed quantitative examination on fusion of the breakpoint cluster region (BCR) gene on chromosome 22 gene in band q11 and Abelson murine leukemia (ABL1) gene on chromosome 9 band q34 (BCR-ABL), 20 patients (54%) achieved major molecular response (MMR), and the remaining 17 (45.9%) had not yet achieved MMR. We found significant relationship between basophils and quantitative BCR-ABL levels (p < 0.01). We didn't find any relationships between these three scores (Eutos, Sokal and Hasford) with treatment response, both hematological and molecular treatment response.

Conclusions: The prevalence of CHR at 3 months was 37.5% meanwhile prevalence of MMR after 12 months was 54%. We found that basophil percent at 3 months after treatment is predictive factor and significantly associated with quantitative BCR-ABL levels (p < 0.05).

Background: Neoadjuvant chemotherapy (NAC), which excludes radiation therapy (RT), has been investigated with the aim of lowering the local recurrence rate and suppressing distant metastasis in patients with rectal cancer, as well as avoiding the adverse events (AEs) of RT.

Aim: To clarify the therapeutic results of NAC for patients with locally advanced mid-low rectal cancer.

Materials and methods: We reviewed the data of 33 patients with stage II or III mid-low rectal cancer who underwent primary tumor resection after NAC between April 2015 and April 2018. We retrospectively analyzed the short-term treatment results, including clinicopathological factors, AEs, and perioperative complications, as well as the long-term treatment results, including the 5-year recurrence-free survival rate (RFS).

Results: The median observation period was 61.5 months (range, 29.6-97.0 months). All patients underwent SOX (S-1 + oxaliplatin) therapy. The most common AEs were thrombocytopenia in five patients (15.1%) and neutropenia in two patients (6.1%). Pathological complete response was achieved in three patients (7.8%). Recurrence was observed in six patients (18.2%), and in each case was a local recurrence event. The 5-year RFS rate was 81.8%. Multivariate analysis using the COX proportional hazards model showed that preoperative lymph node metastasis positivity (p = 0.03) and lymphatic invasion positivity (p < 0.001) were independent prognostic factors.

Conclusion: SOX therapy as preoperative chemotherapy has few adverse events and a low postoperative complication rate, and can be performed relatively safely. Because there were many local recurrences, local control with chemotherapy alone may be insufficient.

Objectives: We investigated the impact of cuff position on the inflow cannula direction and wedge thrombus formation during mid-term HeartWare ventricular assist device (HVAD) support in relatively small patients.

Patients and Methods: Thirty-one patients, including five pediatric patients, underwent HVAD implantation between August 2011 to August 2020 in our institute. Two patients were implanted the HVAD in the true apex and other 29 patients were implanted in the posterior apex. Postoperatively, the direction of the inflow cannula was evaluated by three-dimensional computed tomography, and wedge thrombus was investigated at the time of heart transplantation.

Results: The patients' average body surface area (BSA) was 1.58 ± 0.33 m². Twenty-three had dilated cardiomyopathy and five had ischemic cardiomyopathy. Two patients required the right ventricular assist device (RVAD), and one was weaned from temporary RVAD on postoperative day 20, but another required permanent RVAD implantation using HVAD. The mean support duration was 578 ± 498 days. Fifteen patients successfully bridged to heart transplantation. The survival rate with HVAD support at 3 years was 90.2%. The angle between the inflow cannula and the posterior wall was 34.3 ± 21.6° in patients with posterior apex implantation of HVAD and 5.9 ± 10.2° in those with true apex implantation. Wedge thrombus around the inflow cannula was seen all patients with true apex implantation and one with the posterior apex (8%).

Conclusion: HVAD could be safely used in small patients for mid-term support, and implantation at the posterior apex could be an important option for small patients to prevent wedge thrombus formation during LVAD support.

We report a case of 1-year-old Japanese girl exhibiting severe developmental delay, microcephaly, gastroesophageal reflux, and failure to thrive. Whole exome sequencing revealed likely pathogenic, novel compound heterozygous AARS1 missense variants, inherited from her parents. In cases of severe developmental delay with white matter abnormalities and feeding difficulties, AARS1 abnormalities should be listed as a differential disease.

We describe a case of an 83-year-old man with type 2 diabetes, who had undergone laparoscopy-assisted pyloric 2/3 gastrectomy 7 years ago. Switch from subcutaneous injection of dulaglutide, a glucagon-like protein-1(GLP-1) receptor agonist (GLP-1RA), to administration of oral semaglutide, another GLP-1RA, exacerbated his glycemic status. Because semaglutide is absorbed from the stomach, administration of oral semaglutide to patients after gastrectomy should be avoided even if the surgery is a partial gastrectomy.

The patient was a 72-year-old man who underwent bypass surgery from the ulnar artery to radial artery in the anatomic snuff box with a small saphenous vein graft for right fourth finger gangrene caused by prolonged wound healing due to chronic limb threatening ischemia of the upper extremity. Postoperative transection of the necrotic finger was performed successfully to protect the upper extremity. Cases of chronic limb threatening ischemia of the upper extremity are rare, and there have been limited studies regarding revascularization of the forearm. We present on this case with a review of literature and describe the unique surgical method we performed during the surgery.

A female patient in her 40's noticed a lump in her right breast ten years before her current presentation. One year earlier, the lump began rapidly increasing in size and was accompanied by the development of skin ulcers. Computed tomography revealed a giant mass on the outer side of the right breast. Based on analysis of a biopsy specimen, borderline phyllodes tumor was diagnosed. The patient underwent a simple mastectomy of the right breast with a skin graft. The resected tumor was 6.1 kg in weight, had a maximum diameter of 39 cm, and was pathologically diagnosed as a borderline phyllodes tumor. At postoperative year 2, the patient has had neither a local recurrence nor distant metastasis.

Two-dimensional ^99mTcO₄^- scintigraphy using a gamma camera is widely used as a simple test method that can simultaneously evaluate the morphology and function of the thyroid gland. However, some thyroid diseases require three-dimensional (3D-) morphological evaluation and evaluation of local accumulation. In this study, we used SPECT/CT to verify the quantitative evaluation of autonomously functioning thyroid nodule (AFTN) cases and the measurement of ^99mTcO₄^- uptake rate. In the diagnosis of AFTN, 3D-morphological and functional information was obtained by using ^99mTcO₄^- SPECT/CT. Furthermore, it was clarified that the intake rate obtained by ^99mTcO₄^- SPECT/CT had the same quantitativeness as the conventional method.

The patient described here was a 19-year-old man with no relevant medical history. The patient was treated with antimicrobial agents for pneumonia, and his symptoms improved; however, a shadow persisted in radiographs of the right lower lung field. The patient was referred to our hospital for further examination and treatment. Enhanced computed tomography showed multiple cystic changes with well defined borders in the basement segment of the right lung. An abnormal artery was observed to originate from the celiac artery and returned to the inferior vena cava. We interpreted the symptoms of pulmonary sequestration with abnormal partial pulmonary venous return to be Scimitar syndrome. A surgical intervention was performed thoracoscopically. Scimitar syndrome is a very rare disease. It is important to pay attention to the preoperative cardiac functional assessment and intraoperative vascular treatment for associated pulmonary sequestration.