Significance of early diagnosis and surgical management in treating Mycobacterium immunogenum-related pyogenic extensor tenosynovitis: a case report.

BACKGROUND: Non-tuberculous mycobacteria (NTM) are environmental organisms that are increasingly contributing to human infections. Mycobacterium immunogenum, a variant of NTM discovered in 2001, is a rapidly growing mycobacterium that exhibits multidrug resistance. Reports of infections caused by this organism, particularly tenosynovitis in the musculoskeletal system, are limited. CASE PRESENTATION: A 71-year-old female with vesicular pemphigus, undergoing immunosuppressive therapy, presented with a progressively enlarging tumour on the dorsum of her right hand, along with erythematous papules that extended across her right forearm. The specimens of skin tissues and blood cultures revealed the presence of M. immunogenum. Magnetic resonance imaging evaluation led to the diagnosis of pyogenic extensor tenosynovitis. A multidrug regimen, comprising amikacin and clarithromycin, was initiated, followed by synovectomy. The patient underwent a course of 180 days of antimicrobial therapy and demonstrated no signs of disease recurrence one year after treatment completion. CONCLUSION: Early diagnosis and surgical intervention are crucial to prevent the adverse prognostic implications of pyogenic extensor tenosynovitis caused by M. immunogenum. Effective management requires precise microbial identification and susceptibility testing, necessitating collaborative engagement with microbiological laboratories.

A retrospective study of pediatric renal trauma: A single-center experience in Japan.

OBJECTIVES: To report pediatric renal trauma experiences at the National Center for Child Health and Development in Japan according to the Japanese Association for the Surgery of Trauma (JAST) classification 2008. METHODS: Medical records were retrospectively reviewed for 45 children younger than 18 years old diagnosed with renal trauma from February 2004 to December 2021, regarding details of external causes, treatments, complications, and injury scales according to the JAST classification 2008. The cases who cannot be classified into the JAST classification 2008 will be categorized into our original type 0. RESULTS: There were 24 males and 21 females with a mean age of 8.5 years. Left kidneys were the predominantly affected side. Blunt injury was involved in every case (mainly falls and traffic accidents). Concomitant organ injuries were found in 13 cases. The injury scales were type Ia (13.3%), II (11.1%), IIIa (13.3%), IIIb (24.4%). Type 0 accounted for 37.8%, which were type 0c (congenital anomalies of the kidney and urinary tract without hematoma and/or laceration of kidney parenchyma) at 11.1% and type 0h (only hematuria and normal radiologic finding) at 26.7%. Treatments were blood transfusion, ureteral stenting, nephrostomy, and no nephrectomy. Complications were pseudoaneurysm, hypertension, and infection. CONCLUSIONS: Nearly 38% of cases cannot be classified into the JAST classification 2008, comprising at least two irrelevant types (type 0c and type 0h ). Accordingly, a pediatric renal injury scale should be established to achieve the precise diagnosis and treatments. However, further studies are still needed.

Characteristics of older patients with postmenopausal osteoporosis who developed loss of muscle mass during the COVID-19 pandemic - a case-control study.

BACKGROUND: Under the restriction of social activities during the coronavirus disease 2019 (COVID-19) pandemic, there was concern about the loss of muscle mass due to a decrease in physical activity for the elderly. The purpose of this study was to investigate the characteristics of older patients with postmenopausal osteoporosis who developed loss of muscle mass during the COVID-19 pandemic in Japan. METHODS: A total of 54 patients with postmenopausal osteoporosis were evaluated in this study. Whole-body dual-energy X-ray absorptiometry was performed pre- and post-COVID-19 pandemic to measure trunk and lower limb muscle mass. At the time of the post-COVID-19 pandemic, we conducted a survey to compare lifestyle before pandemic (the frequency of going out, the frequency of meeting acquaintances or families living apart, regular exercise habits, walking time, family structure), and comorbidities between the muscle mass loss (ML) group and the muscle mass maintenance (MM) group. The ML group consisted of patients with at least a 5% decrease in lower limb muscle mass or trunk muscle mass. RESULTS: A significant difference was found only for the family structure (P = 0.0279); in the ML group, those living alone were the largest group, while in the MM group they were the smallest group. CONCLUSIONS: The ML group was significantly more likely to live alone than the MM group. The current study showed that loss of muscle mass was more common in patients living alone.

Potential of whole-body dual-energy X-ray absorptiometry to predict muscle size of psoas major, gluteus maximus and back muscles.

BACKGROUND: Measurement of trunk muscle cross-sectional area (CSA) using axial magnetic resonance imaging (MRI) is considered clinically meaningful for understanding several spinal pathologies, such as low back pain and spinal sagittal imbalance. However, it remains unclear whether trunk muscle mass (TMM) measured using dual-energy X-ray absorptiometry (DXA) can predict the trunk muscle CSA. The aim of this study is to determine if DXA-derived TMM is associated and predicts with CSA of paraspinal muscles and gluteus maximus measured using MRI in healthy volunteers. METHODS: A total of 48 healthy volunteers underwent whole-body DXA and MRI of the spinopelvic region. The CSA of the psoas major, back muscles, and gluteus maximus were measured on axial MRI. Correlations and linear regressions between the TMM measured using DXA and the CSA of each musculature were investigated. RESULTS: There was a weak correlation between TMM and CSA of the psoas major in men (r = 0.39, P = 0.0678), and the linear regression was y = 301.74x - 401.24 (R2 = 0.2976, P = 0.0070). A moderate correlation was found in women (r = 0.58, P = 0.0021), and the linear regression was y = 230.21x - 695.29 (R2 = 0.4445, P = 0.0003). Moderate correlations were observed between TMM and CSA of the back muscles in both men (r = 0.63, P = 0.0012) and women (r = 0.63, P = 0.0007), the linear regression was y = 468.52x + 3688.5 (R2 = 0.5505, P < 0.0001) in men and y = 477.39x + 2364.1 (R2 = 0.564, P < 0.0001) in women. There was a strong correlation between TMM and CSA of the gluteus maximus in men (r = 0.72, P < 0.0001), and the linear regression was y = 252.69x - 880.5 (R2 = 0.6906, P < 0.0001). A moderate correlation was found in women (r = 0.69, P < 0.0001), and the linear regression was y = 230.74x - 231.32 (R2 = 0.6542, P < 0.0001). CONCLUSIONS: The DXA-derived TMM was able to predict the CSA of the psoas major, back muscles, and gluteus maximus, and significantly correlated with the CSA of the back muscles and gluteus maximus. It might be a safer and cheaper alternative for evaluating the size of the back muscles and gluteus maximus.

Posterior lumbar fusion surgery doesn't change sexual activities in patients with lumbar degenerative disease: an observational study.

BACKGROUND: There are few studies about sexual function in the patient with posterior lumbar spinal fusion for degenerative lumbar disease. The aim of this study is to investigate sexual activities in patients with lumbar degenerative disease before and after lumbar fusion surgery. METHODS: We recruited 35 patients who underwent lumbar spinal fusion at the age of 55 years or younger. They were 17 men and 18 women with a mean age of 47.4 years. After informed consent, the patients were asked to complete anonymous questionnaire concerning sexual desire, activity, and satisfaction before and after surgery. RESULTS: In the presick period, 69% of the patients had sexual desire, and 79% achieved satisfaction during sexual activity. Lumbar degenerative disease decreased sexual desire and frequency of sexual activity in 40%, and 74% respectively. Before surgery, satisfaction in sexual activities decreased in 53%, and 55% of the patients felt discomfort during sexual activity. Adjustment in sexual position was required in 44% of man and 54% of woman. After surgery, Sexual desire, frequency of sexual activity and satisfaction did not regain after surgery in 94%, 93% and 92%, respectively. Those who did not feel discomfort after surgery was significantly lower VAS in both low back pain and leg pain than the patients felt discomfort (low back pain; p = 0.024, leg pain; p = 0.046). CONCLUSION: This study demonstrated that lumbar degenerative diseases decreased sexual desire, frequency of sexual activity and satisfaction, and little of the patients regained their sexual activities after posterior lumbar fusion surgery in the middle-aged patients.

Pre-existing adjacent level foraminal stenosis does not affect the outcome of a single level lumbar interbody fusion.

BACKGROUND: The aim of this study was to investigate whether or not pre-existing asymptomatic neuroforaminal stenosis adjacent to the fusion level develops adjacent segment disease (ASD) after single-level lumbar interbody fusion. SUMMARY AND BACKGROUND DATA: Risk factors of ASD after spinal fusion have been well investigated, but there have been few studies focused on the relationship between ASD and pre-existing asymptomatic neuroforaminal stenosis. METHODS: A total of 302 patients who had undergone a single-level lumbar interbody fusion were reviewed at a minimum of 2 year follow-up. They were 109 men and 193 women with a mean age of 68.8 years. Follow-up periods was averaged 53.5 months. ASD was defined as neurological deterioration related to adjacent segment pathologies which required an additional surgery. Based on the pathologies, patients were divided into three categories: ASD due to foraminal stenosis (ASD-FS), ASD due to central stenosis (ASD-CS), and ASD due to herniated disc (ASD-HD). Measured variables were age, gender, diagnosis, BMI, decompression procedures at adjacent segments, preoperative anterior/posterior slip, asymptomatic neuroforaminal stenosis, facet tropism, and postoperative spinopelvic parameters. RESULTS: Thirty-eight patients (12.6%) developed ASD. There were 15 patients with ASD-FS, 18 patients with ASD-CS, and five patients with ASD-HD. Lumbar lordosis (LL) and sacral slope (SS) were significantly smaller and pelvic tilt (PT) was significantly larger in ASD-FS. Asymptomatic neuroforaminal stenosis was detected preoperatively in 33.3% of the ASD-FS group, and 18.6% of non-ASD group; the incidence was not significantly different. CONCLUSIONS: Adjacent-level neuroforaminal stenosis was not a significant risk of ASD after single-level lumbar interbody fusion, and might not need to be fused if asymptomatic.

Association between obesity and ossification of spinal ligaments in 622 asymptomatic subjects: a cross-sectional study.

INTRODUCTION: Previous studies on patients with symptoms of spinal ligament ossification, including ossification of the posterior longitudinal ligament (OPLL) and ligamentum flavum (OLF), have not clarified whether obesity is a cause or consequence of these diseases and were limited by selection bias. Thus, we investigated the association between obesity and the prevalence of spinal ligament ossification in randomly selected asymptomatic subjects. MATERIALS AND METHODS: Between April 2020 and March 2021, 622 asymptomatic Japanese subjects who underwent computed tomography of neck to pelvis for medical check-up purposes were included. All subjects were divided into the following three groups: normal weight (body mass index [BMI] < 25 kg/m2), obese I (25 ≤ BMI < 30 kg/m2), and obese II (BMI ≥ 30 kg/m2). The relationship between factors affecting the presence of each spinal ligament ossification was evaluated using multivariate logistic regression analysis. RESULTS: The proportion of subjects with thoracic OPLL was significantly higher in the obese II group than in the other two groups (vs. normal weight, P < 0.001; vs. obese I, P < 0.001). BMI was associated with the prevalence of OLF, cervical OPLL, thoracic OPLL, and ossification of the anterior longitudinal ligament (OALL). BMI was most significantly associated with the prevalence of thoracic OPLL (ß, 0.28; 95% confidence interval, 0.17-0.39). CONCLUSION: BMI was associated with the prevalence of OALL, cervical OPLL, thoracic OPLL, and OLF in asymptomatic subjects, suggesting that obesity is associated with the development of heterotopic ossification of the spinal ligaments.

Early administration of cyclosporine may reduce the incidence of cytokine release syndrome after HLA-haploidentical hematopoietic stem-cell transplantation with post-transplant cyclophosphamide.

Cytokine release syndrome (CRS), occurring in more than 70% of HLA-haploidentical hematopoietic stem-cell transplantations with post-transplant cyclophosphamide (PT/CY-haplo), can lead to hemodynamic instability and worsen clinical outcomes. A calcineurin inhibitor is initiated after cyclophosphamide administration in the commonly used PT/CY regimens. Here, we conducted a phase I/II, prospective, single-center trial of PT/CY-haplo to evaluate the safety and efficacy of cyclophosphamide on days 3 and 5 along with cyclosporin and mycophenolate mofetil started from day - 1. Thirty-five adults with hematologic malignancies were enrolled. Myeloablative and reduced-intensity conditioning were used in 25 and 10 patients, respectively. Graft sources were bone marrow in 11 patients and mobilized peripheral blood stem cells in 24 patients. Disease-free survival on day 100, the primary endpoint, was 86% (95% confidence interval (CI), 69-94), which was over the predefined threshold of 50%. Unexpectedly, only 20% (95% CI, 8.4-37) of patients developed fever of > 38 °C early after graft infusion, all CRS grade 1, and all of which resolved just after cyclophosphamide administration. The cumulative incidences of grades II-IV acute graft-versus-host disease (GVHD), III-IV acute GVHD, and moderate-severe chronic GVHD were 23% (95% CI, 11-38), 6% (95% CI, 1-17), and 11% (95% CI, 4-25), respectively. The 3-year overall survival rate was 49% (95% CI, 31-64). Our results suggest that administration of cyclosporine and mycophenolate mofetil prior to PT/CY can reduce the frequency and severity of CRS without increasing GVHD. UMIN Clinical Trial Registry numbers: 000006631 and 000015694.

Transfusion-associated circulatory overload and high blood pressure: A multicentre retrospective study in Japan.

BACKGROUND: Transfusion-associated circulatory overload (TACO) is an adverse reaction associated with a high risk of mortality. The actual incidence of TACO and hypertension associated with transfusion in Japan is unknown. METHODS: A multicentre retrospective observational study was conducted across 23 institutions during the 1-year period of 2016. Patients were included if they developed TACO or their blood pressure (either systolic or diastolic) increased by at least 30 mmHg during the transfusion. TACO was confirmed by the primary physicians and transfusion medicine teams and recorded in the data on passive surveillance, and additional data were extracted from electronic medical records. RESULTS: In our patient cohort of 31 384 patients who underwent transfusion, the incidence of TACO and hypertension was 0·03% and 0·2%, respectively. However, 43% of the participating institutions didn't report any cases. When comparing risk factors between the TACO and hypertension groups, there were significant differences in comorbidities, such as abnormal findings on chest x-ray. Significant differences between the two groups were observed post-transfusion pulse rate, body temperature and oxygen saturation (P < 0·01). In the group of patients with hypertension, the level of BNP increased significantly after transfusion in 45% (5/11) of the patients. We identified 4 patients in the hypertension group who met the new ISBT's TACO criteria. CONCLUSION: Our study suggests that more attention should be given to TACO in Japan, particularly in terms of improving surveillance systems. For the early diagnosis of TACO, it is crucial to carefully monitor vital signs including blood pressure.

Replacing mandibular central incisors with a direct resin-bonded fixed dental prosthesis by using a bilayering composite resin injection technique with a digital workflow: A dental technique.

A straightforward technique is presented for an interim or short-term definitive esthetic replacement of missing anterior teeth requiring no tooth preparation. Composite resins are injected into transparent silicone indices fabricated from 3-dimensional-printed casts of a digital waxing. The dentin core is formed of a durable short fiber-reinforced injectable composite resin and veneered with an enamel-shade composite resin for enhanced esthetics. Besides being noninvasive, this technique is more straightforward than traditional options, reducing chair time while providing an accurate outcome.

[Acquired platelet dysfunction with severe bleeding tendency in triple-negative myelofibrosis].

A 50-year-old man demonstrated markedly increased number of white blood cells, anemia, severe splenomegaly, and bleeding tendency. Bone marrow analysis revealed remarkable hypercellularity; dysplasia in multilineage cells, including megakaryocytes; and fibrosis. He was eventually diagnosed with triple-negative myelofibrosis. A massive hematoma developed at the bone marrow biopsy site. A similar episode recurred after the second bone marrow biopsy. The von Willebrand factor and other coagulation factor activities were within normal ranges. Platelet aggregation analyses demonstrated highly impaired aggregation induced by ADP, collagen, and epinephrine. Treatment with hydroxyurea and ruxolitinib, a JAK inhibitor, was ineffective, and he eventually died on day 144 after hospitalization. Acquired platelet dysfunction uncommonly occurs in patients with myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN), without precise elucidation of the frequency and underlying mechanism. The onset of bleeding tendency in the current patient suggested that platelet dysfunction may be caused by somatic genetic events. Here, we discuss the mechanisms of acquired platelet dysfunction in MDS or MPN with a literature review.

A Japanese multi-institutional collaborative study of antigen-positive red blood cell (RBC) transfusions in patients with corresponding RBC antibodies.

BACKGROUND AND OBJECTIVES: It is sometimes difficult to obtain antigen-negative red blood cells (RBCs) for patients with antibodies against RBCs. However, the frequency and severity of the adverse reactions have not been well elucidated. Here, we conducted a multi-institutional collaborative study to clarify the background, frequency and clinical significance of antigen-positive RBC transfusions to patients with the respective antibodies. MATERIALS AND METHODS: The survey included the background of patients, antigens on RBCs transfused, total amount of antigen-positive RBCs transfused, results from antibody screen and direct antiglobulin tests, specificity of antibodies, adverse reactions and efficacies. All antibodies were surveyed regardless of their clinical significance. RESULTS: In all, 826 cases containing 878 antibodies were registered from 45 institutions. The main reasons for antigen-positive RBC transfusions included 'negative by indirect antiglobulin test' (39%) and 'detection of warm autoantibodies' (25%). In 23 cases (3% of total), some adverse reactions were observed after antigen-positive RBC transfusion, and 25 antibodies (9 of 119 clinically significant and 16 of 646 insignificant antibodies) were detected. Non-specific warm autoantibodies were detected in 9 cases, anti-E in 5 cases, 2 cases each of anti-Lea , anti-Jra or cold alloantibodies, and 1 case each of anti-Dib , anti-Leb or anti-P1. Other antibodies were detected in 2 further cases. Five (22%) of these 23 cases, who had anti-E (3 cases) or anti-Jra (2 cases), experienced clinically apparent haemolysis. CONCLUSIONS: Adverse reactions, especially haemolysis, were more frequently observed in cases with clinically significant antibodies than those with clinically insignificant antibodies (P < 0·001).

Ten years change in post-fracture care for hip fracture patients.

INTRODUCTION: This multicenter, retrospective study aimed to clarify the changes in postoperative care provided by orthopaedic surgeons after hip fractures and clarify the incidence of secondary fractures requiring surgery. MATERIALS AND METHODS: Subjects were patients with hip fracture treated surgically in seven hospitals during the 10-year period from January 2008 to December 2017. Data on patient demographics, comorbidities, preoperative and postoperative osteoporosis treatments, and secondary fractures were collected from the medical records. RESULTS: In total, 4764 new hip fractures in 982 men and 3782 women (mean age: 81.3 ± 10.0 years) were identified. Approximately 10% of patients had a history of osteoporosis drug treatment and 35% of patients received postoperative drug treatment. The proportion of patients receiving postoperative drug therapy increased by approximately 10% between 2009 and 2010, 10% between 2010 and 2011, and 10% between 2011 and 2013. Although the rate of secondary fractures during the entire period and within 3 years decreased from 2011, the rate of secondary fracture within 1 year remained at around 2% every year. CONCLUSIONS: The approval of new osteoporosis drugs and the establishment of osteoporosis liaison services have had a positive effect on the use of postoperative drug therapy in the orthopedic field. Our finding that the rate of secondary fracture within 1 year of the initial fracture remained around 2% every year, despite improvements in postoperative drug therapy, suggests that both rehabilitation for preventing falls and early postoperative drug therapy are essential to prevent secondary fractures.

[Haploidentical transplantation from a related donor as first-line treatment for fulminant aplastic anemia].

Immunosuppressive therapy (IST) is the first-line treatment for young patients with severe aplastic anemia (AA) when a human leucocyte antigen (HLA)-matched related donor (MRD) is unavailable. Fulminant AA (FAA) is defined as AA with a complete absence of neutrophils at presentation and no response to granulocyte-colony stimulating factor (G-CSF) treatment. Here we report a 38-year-old male FAA patient who underwent allogeneic stem cell transplantation from an HLA haplotype-mismatched donor as first-line treatment. The patient had no remarkable disease history and was referred to our hospital because of a peritonsillar abscess and severe pancytopenia. Bone marrow biopsy revealed marked hypocellularity without dysplasia. His neutrophil count remained 0.0×109/l following G-CSF administration, and he was diagnosed with FAA. His siblings were not MRDs, but his sister had haploidentical HLAs. After administering a conditioning regimen, the patient received a transplant of peripheral blood stem cells donated by his sister. Neutrophil engraftment was observed on post-transplant day 16, and he experienced acute graft-versus-host disease (grade I, skin stage 1), but no other complications were observed. Hematopoietic stem cell transplantation from an HLA haplotype-mismatched related donor may be a viable option for first-line treatment of FAA when an MRD is unavailable.

[Philadelphia chromosome-positive acute lymphoblastic leukemia complicated by bone marrow necrosis during consolidation chemotherapy].

A 46-year-old man who had previously undergone open surgery for renal cell carcinoma (RCC) developed Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph-ALL). After the induction therapy, he achieved complete molecular remission. However, fever and bilateral buttock pain continued during the consolidation therapy. 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) showed FDG accumulation in both iliac bones and in the sacrum; however, no causative diseases, including relapse of Ph-ALL and RCC, were detected. Iliac bone marrow biopsy revealed bone marrow necrosis (BMN), the etiology of which was presumed to be the leukemia itself and the therapeutic response to chemotherapy. Fever resolution and buttock pain alleviation were observed over the next months. We observed diffuse fibrosis in the bone marrow at day 162 and day 364 after cord blood transplantation. Moreover, the FDG accumulation was significantly reduced on PET-CT. BMN is not widely recognized despite its potential association with hematologic malignancies. Additional cases of BMN should be reviewed to clarify BMN etiology and clinical features.

Genetic evidence implies that primary and relapsed tumors arise from common precursor cells in primary central nervous system lymphoma.

Primary central nervous system lymphoma (PCNSL) is a rare subtype of lymphoma that arises within the brain or the eyes. PCNSL recurs within the central nervous system (CNS) in most relapsed cases, whereas extra-CNS relapse is experienced in rare cases. The present study aimed at identifying the presence of common precursor cells (CPC) for primary intra- and relapsed extra-CNS tumors, and further assessing the initiating events in bone marrow (BM). Targeted deep sequencing was carried out for five paired primary intra- and relapsed extra-CNS tumors of PCNSL. Two to five mutations were shared by each pair of intra- and extra-CNS tumors. In particular, MYD88 mutations, L265P in three and P258L in one, were shared by four pairs. Unique somatic mutations were observed in all five intra-CNS tumors and in four out of five extra-CNS tumors. Remarkably, IgH clones in the intra- and the extra-CNS tumors in two pairs were distinct from each other, whereas one pair of tumors shared identical monoclonal IgH rearrangement. In a cohort of 23 PCNSL patients, L265P MYD88 mutations were examined in tumor-free BM mononuclear cells (MNC) in which the PCNSL tumors had L265P MYD88 mutations. L265P MYD88 mutations were detected by a droplet digital PCR method in nine out of 23 bone marrow mononuclear cells. These results suggest that intra- and extra-tumors are derived from CPC with MYD88 mutations in most PCNSL, arising either before or after IgH rearrangement. The initiating MYD88 mutations may occur during B-cell differentiation in BM.

Necessity of performing voiding cystourethrography for children with unilateral multicystic dysplastic kidney.

BACKGROUND: The purpose of this study was to resolve the clinical question as to whether all patients with unilateral multicystic dysplastic kidney (MCDK) should receive voiding cystourethrography (VCUG). METHODS: This is a retrospective study using cross-sectional analysis. Seventy-five children with unilateral MCDK were enrolled, excluding patients with other genetic or chromosome abnormalities, spinal cord diseases, or anal atresia. We reviewed their records from medical charts and calculated risk factors for abnormal VCUG using multivariate logistic regression analysis. RESULTS: Abnormal VCUG findings were present in 24 of 75 patients (32.0%), specifically, vesicoureteral reflux (VUR) in 8 (10.6%), including high-grade VUR in 2 (2.7%), and only lower urinary tract or bladder disease in 16 (21.3%). In multivariate analysis, only abnormal findings by ultrasonography was an independent risk factor for abnormal VCUG findings with statistical significance in multivariate analysis (OR 6.57; 95% CI 1.99-26.26; P = 0.002). When we excluded five patients who showed similar findings by ultrasonography and VCUG, abnormal findings by ultrasonography were again calculated as an independent risk factor (OR 4.44; 95% CI 1.26-28.42; P = 0.02). Sensitivity, specificity, positive predictive value, and negative predictive value of abnormal findings by ultrasonography to predict urologic anomalies by VCUG in these children were 83%, 59%, 49%, and 88%, respectively. Two children required a third ultrasonography to detect abnormal findings. CONCLUSIONS: We can select, using only abnormal findings by ultrasonography, children with unilateral MCDK who should undergo VCUG. We would also like to emphasize that ultrasonography should be performed repeatedly to detect congenital anomalies of the urinary tract.

[Successful management of primary immune thrombocytopenia with romiplostim during open heart surgery in a hemodialysis patient].

A 66-year-old male undergoing maintenance hemodialysis presented with mild thrombocytopenia. He also had aortic valve stenosis and required aortic valve replacement. In addition, he required anticoagulation therapy with warfarin because of chronic subclavian artery occlusion. He was eventually diagnosed with immune thrombocytopenic purpura (ITP), although there were no bleeding tendencies. The patient was preoperatively treated with thrombopoietin receptor agonist (romiplostim®) because of the risk of bleeding complication during cardiac surgery. The platelet count rapidly increased with low-dose romiplostim, and no thrombotic complication occurred. During surgery, no significant bleeding complications were observed. This report suggests that romiplostim is a useful treatment option for the management of bleeding complication during cardiac surgery in a hemodialysis patient with ITP.

[Fatal exacerbations of chronic active Epstein-Barr virus infection subsequent to cytotoxic chemotherapy].

Chronic active Epstein-Barr virus infection (CAEBV) is critical owing to lethal complications such as hemophagocytic lymphohistiocytosis (HLH), multiple organ failure, and malignant lymphoma. Here we present two cases of CAEBV who developed rapid and life-threatening disease progression after cytotoxic chemotherapy. Case 1: In a 34-year-old male, CAEBV recurred after 4-month remission obtained by initial therapy with etoposide, cyclosporine, and prednisolone. Accordingly, cord blood transplantation was planned. A day after administering high-dose melphalan as the conditioning, he developed respiratory failure, pancytopenia, and hyperferritinemia. He died 3 days later. Case 2: A 53-year-old female attained remission after initial therapy for CAEBV. After 1 month, she relapsed, and high-dose cytarabine (HDAC) was administered. A day after HDAC administration, she suddenly developed respiratory failure, which was followed by multiple organ failure. She died 3 days later. Thus, planned strategy for prompt allogeneic hematopoietic stem cell transplantation is necessary to prevent disease progression and control cytokinemia before cytotoxic chemotherapy for CAEBV.

Clinical significance of disease-specific MYD88 mutations in circulating DNA in primary central nervous system lymphoma.

Recent sequencing studies demonstrated the MYD88 L265P mutation in more than 70% of primary central nervous system lymphomas (PCNSL), and the clinical significance of this mutation has been proposed as diagnostic and prognostic markers in PCNSL. In contrast, mutational analyses using cell-free DNAs have been reported in a variety of systemic lymphomas. To investigate how sensitively the MYD88 L265P mutation can be identified in cell-free DNA from PCNSL patients, we carried out droplet digital PCR (ddPCR) and targeted deep sequencing (TDS) in 14 consecutive PCNSL patients from whom paired tumor-derived DNA and cell-free DNA was available at diagnosis. The MYD88 L265P mutation was found in tumor-derived DNA from all 14 patients (14/14, 100%). In contrast, among 14 cell-free DNAs evaluated by ddPCR (14/14) and TDS (13/14), the MYD88 L265P mutation was detected in eight out of 14 (ddPCR) and in 0 out of 13 (TDS) samples, implying dependence on the detection method. After chemotherapy, the MYD88 L265P mutation in cell-free DNAs was traced in five patients; unexpectedly, the mutations disappeared after chemotherapy was given, and they remained undetectable in all patients. These observations suggest that ddPCR can sensitively detect the MYD88 L265P mutation in cell-free DNA and could be used as non-invasive diagnostics, but may not be applicable for monitoring minimal residual diseases in PCNSL.