Allogeneic stem cell transplantation for inherited metabolic disorders: 35 years' experience at a single institution.

Hematopoietic stem cell transplants for inherited metabolic disorders performed at Tokai University Hospital between June 5, 1986, and May 28, 2021, were analyzed and compared between the period before 2007 and the period from 2007 onward based on availability of medical resources. Transplants were performed for 38 patients with mucopolysaccharidosis, 33 with adrenoleukodystrophy, and 16 with another disorder. Before 2007, oral busulfan-based regimens were mainly used. From 2007 onward, intravenous busulfan-based regimens or 4 Gy of thoracoabdominal irradiation (TAI), fludarabine, and melphalan (Mel)/treosulfan were adopted. Between 2002 and 2010, adrenoleukodystrophy was treated with 12 Gy of TAI and Mel. HLA-identical sibling bone marrow was used in 43% of cases before 2007 and 15% from 2007 onward, while alternative donors were selected for other transplants. Overall survival and event-free survival (EFS) before 2007 and from 2007 onward were 76% and 62%, and 97% and 85%, respectively (P = 0.006 and 0.017). Transplant era predicted superior overall survival and EFS, while myeloablative conditioning also predicted EFS. The incidence of primary graft failure decreased from 2007 onward, especially in cord blood transplant when 4 Gy of TAI with 150 mg/m2 fludarabine and 180 mg/m2 Mel or 42 g/m2 treosulfan were used as conditioning.

Visualising cervical lymph nodes in jaw osteonecrosis using diffusion-weighted imaging.

OBJECTIVES: This study aimed to apply diffusion-weighted imaging to the evaluation of cervical lymph nodes affected by medication-related osteonecrosis of the jaw (MRONJ). METHODS: This retrospective study analysed the diffusion-weighted imaging data of 70 patients with or without MRONJ (Stages 0-3). The mean apparent diffusion coefficient (ADC) values of the submandibular (level IB) and superior internal jugular (level IIA) nodes in the control and MRONJ groups were calculated, and differences were analysed using the Mann-Whitney test. Moreover, receiver operating characteristic (ROC) curve analysis was performed to evaluate the ability of ADC values to predict lymph nodes that were affected by MRONJ. p < 0.05 was considered statistically significant. RESULTS: The median [interquartile range] (×10-3 mm2/s) of level IB was 0.74 [0.7-0.81] and 0.93 [0.84-1.09] and that of level IIA was 0.79 [0.76-0.85] and 0.97 [0.84-1.06] in the control and MRONJ groups respectively. ROC analysis revealed that the ADC value had excellent ability to discriminate between the control and MRONJ groups. CONCLUSIONS: The study findings indicate that diffusion-weighted imaging can contribute to differentiation of MRONJ from other cervical lymph node diseases and facilitate early detection of MRONJ.

Quantitative assessment of mandibular bone marrow using computed tomography texture analysis for detect stage 0 medication-related osteonecrosis of the jaw.

PURPOSE: Medication-related osteonecrosis of the jaw (MRONJ) is a serious complication of treatment with bisphosphonates or antiangiogenic inhibitors. MRONJ has four stages (0-3); however, stage 0 MRONJ is difficult to detect using computed tomography (CT). This study aimed to quantitatively assess the mandibular bone marrow using texture analysis to detect stage 0 MRONJ from CT images. METHODS: This retrospective study included 25 patients with stage 0 MRONJ who had a history of treatment with bisphosphonates and underwent CT and magnetic resonance imaging (MRI). The mandibular bone marrow with abnormal signals (T1-weighted imaging: low, T2-weighted imaging: low or high, short-tau inversion recovery: high) on MRI, and no qualitative characteristic CT and oral findings indicative of osteonecrosis (exposed bone, sequestrum, periosteal reaction, and osteolysis) was identified as 0 MRONJ. Texture features of the bone marrow of the mandible with MRONJ and the contralateral, normal mandibular bone marrow were extracted using an open-access software, namely, LIFEx. The volumes of interest (VOIs) were manually placed on CT images by tracing the bilateral mandibular bone marrow regions, excluding the teeth, mandibular canal, and cortical bone. Thirty-seven texture features were extracted from each VOI. RESULTS: Six gray-level run length matrix features and four gray-level zone length matrix features exhibited significant differences between mandibular bone marrow with and without MRONJ. CONCLUSIONS: CT was able to quantitatively assess texture features of normal mandibular bone marrow and that with MRONJ. Texture analysis may be useful as a new method for detecting stage 0 MRONJ using CT.

Engineering Nanowire-Mediated Cell Lysis for Microbial Cell Identification.

Researchers have demonstrated great promise for inorganic nanowire use in analyzing cells or intracellular components. Although a stealth effect of nanowires toward cell surfaces allows preservation of the living intact cells when analyzing cells, as a completely opposite approach, the applicability to analyze intracellular components through disrupting cells is also central to understanding cellular information. However, the reported lysis strategy is insufficient for microbial cell lysis due to the cell robustness and wrong approach taken so far ( i. e., nanowire penetration into a cell membrane). Here we propose a nanowire-mediated lysis method for microbial cells by introducing the rupture approach initiated by cell membrane stretching; in other words, the nanowires do not penetrate the membrane, but rather they break the membrane between the nanowires. Entangling cells with the bacteria-compatible and flexible nanowires and membrane stretching of the entangled cells, induced by the shear force, play important roles for the nanowire-mediated lysis to Gram-positive and Gram-negative bacteria and yeast cells. Additionally, the nanowire-mediated lysis is readily compatible with the loop-mediated isothermal amplification (LAMP) method because the lysis is triggered by simply introducing the microbial cells. We show that an integration of the nanowire-mediated lysis with LAMP provides a means for a simple, rapid, one-step identification assay (just introducing a premixed solution into a device), resulting in visual chromatic identification of microbial cells. This approach allows researchers to develop a microfluidic analytical platform not only for microbial cell identification including drug- and heat-resistance cells but also for on-site detection without any contamination.

Laboratory and clinical features of abnormal macroenzymes found in human sera.

We report the analysis of unusual macroenzymes, performed in our laboratory, and review the relevant literature. In particular, we focused on macro AST, macroamylase, macro LD and macro CK. Macroenzymes are seen in healthy subjects, but can also be related to disease; thus, accurate detection is useful in day-to-day clinical practice. The macroenzyme is thought to be a specific antigen-antibody complex from the following findings: (1) the complex could be dissociated under acidic pH levels; (2) binding specificity of immunoglobulin in the complex was observed; (3) the binding site of immunoglobulin in the complex was Fab portion; and (4) the maternal IgG involved with macroenzyme was transferred to her children. This article is part of a Special Issue entitled: Medical Proteomics.

Magnetic Properties of the Ferromagnetic Shape Memory Alloys Ni50+xMn27-xGa23 in Magnetic Fields.

Thermal strain, permeability, and magnetization measurements of the ferromagnetic shape memory alloys Ni50+xMn27-xGa23 (x = 2.0, 2.5, 2.7) were performed. For x = 2.7, in which the martensite transition and the ferromagnetic transition occur at the same temperature, the martensite transition starting temperature TMs shift in magnetic fields around a zero magnetic field was estimated to be dTMs/dB = 1.1 ± 0.2 K/T, thus indicating that magnetic fields influences martensite transition. We discussed the itinerant electron magnetism of x = 2.0 and 2.5. As for x = 2.5, the M4 vs.B/M plot crosses the origin of the coordinate axis at the Curie temperature, and the plot indicates a good linear relation behavior around the Curie temperature. The result is in agreement with the theory by Takahashi, concerning itinerant electron ferromagnets.