Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of various autoantibodies and deposition of immune complexes on tissues. Acquired thrombotic ...thrombocytopenic purpura (TTP) is a life-threatening hematological disorder that rarely develops in SLE, mainly caused by inhibitory or clearing autoantibody against ADAMTS13. Although B cells play critical roles in the pathogenesis of two diseases, the role of B-cell depletion therapy using rituximab (RTX), a chimeric monoclonal antibody targeting CD20, in the management of TTP associated with SLE remains unclear. We present a 27-year-old woman who manifested TTP and nephritis simultaneously at diagnosis of SLE. This patient successfully responded to high-dose glucocorticoids combined with plasma exchange, and early administration of RTX-induced sustained remission of TTP without relapse over 16 months. This literature review in light of our case demonstrates relationship between early intervention with RTX and better treatment response despite the degree of ADAMTS13 activity. Moreover, we discuss the clinical features in TTP associated with SLE, risk factors for the development of TTP in SLE, and possible outcomes based on RTX dose. It is important to consider upfront RTX as a promising treatment strategy for SLE-associated secondary TTP to improve short-term response and long-term prognosis.
Based on the transmission line theory, modal transformation is applied to a balanced two-conductor line connected to a signal source and a load, which yields an equivalent circuit expressed in ...differential and common modes. This newly developed equivalent circuit clearly shows that the common-mode currents on a balance two-conductor line are generated by (1) direct injection of the common-mode current by a connected RF source; (2) multiple mode conversions by the source and load; and (3) multiple reflections of the modal currents at the source and load. The effects of branch lines connected to the feeder line are also investigated, focusing on generation of the common-mode current. It is concluded that the common-mode currents are also generated by (4) series connection of a branch line. Particularly, in the case of a switching branch line, the connection of a feeder line makes the branch line imbalanced, resulting in the common-mode current in the branch line. The aforementioned theoretical findings are compared with computer simulation using the method of moments with the numerical electromagnetic code (NEC2).
Conducted disturbance measurements on the mains cable are seriously affected by the equipment under test port impedance of the artificial mains network (AMN). This impedance is called the AMN ...impedance. In order to measure the AMN impedance, an ac-coaxial adapter has to be used to connect the AMN to impedance measuring equipment. Accordingly, the adapter has to be accurately calibrated. This paper describes theoretical and experimental investigations on the characteristics of the conventional adapter calibration methods, i.e., open/short termination method, electrical length correction method, and Kriz's method. In addition, this paper proposes a novel calibration method named S-parameter method. Uncertainties are also evaluated for each calibration method. It is found that the open/short termination method, Kriz's method, and the S-parameter method yield almost the same corrected value of the AMN impedance.
Hereditary spastic paraplegia (HSP) is one of the most genetically heterogeneous neurodegenerative disorders characterized by progressive spasticity and pyramidal weakness of lower limbs. Because >30 ...causative genes have been identified, screening of multiple genes is required for establishing molecular diagnosis of individual patients with HSP. To elucidate molecular epidemiology of HSP in the Japanese population, we have conducted mutational analyses of 16 causative genes of HSP (L1CAM, PLP1, ATL1, SPAST, CYP7B1, NIPA1, SPG7, KIAA0196, KIF5A, HSPD1, BSCL2, SPG11, SPG20, SPG21, REEP1 and ZFYVE27) using resequencing microarrays, array-based comparative genomic hybridization and Sanger sequencing. The mutational analysis of 129 Japanese patients revealed 49 mutations in 46 patients, 32 of which were novel. Molecular diagnosis was accomplished for 67.3% (33/49) of autosomal dominant HSP patients. Even among sporadic HSP patients, mutations were identified in 11.1% (7/63) of them. The present study elucidated the molecular epidemiology of HSP in the Japanese population and further broadened the mutational and clinical spectra of HSP.
Chilaiditi syndrome is a rare pathophysiology in which the colon or other organs are interposed between the diaphragm and liver, and respiratory or digestive symptoms sometimes manifest. Although ...there have been some cases of Chilaiditi syndrome complicating neuromuscular disorders, none have described resulting respiratory or digestive symptoms.
Our patient was a 20-year-old man with DMD who had been receiving noninvasive positive-pressure ventilation during the night. He experienced respiratory distress when changing from a supine to sitting position. Ventilator adjustment did not relieve the respiratory distress. Abdominal computed tomography revealed marked constipation and interposition of the transverse colon between the diaphragm and liver, indicating Chilaiditi syndrome. The right side of the diaphragm was elevated by the interposed transverse colon when the respiratory distress was present on chest radiograph, but not when symptoms were absent. The patient was diagnosed with platypnea-orthodeoxia attributed to Chilaiditi syndrome. The respiratory distress was improved by the relief of constipation, in addition to the usage of the ventilator throughout the day.
The rare symptoms and pathophysiology of DMD complicated by Chilaiditi syndrome are reported and discussed herein.
Theoretical and experimental investigations were carried out on the characteristics of the shunt-type asymmetric artificial network (AAN). The findings are as follows: 1) The theoretical formulas for ...the differential- and common-mode impedances as well as the longitudinal conversion loss (LCL) were derived for the shunt-type AAN in terms of the AAN circuit parameters. 2) The common-mode disturbance voltage estimated from the measured voltage is not proportional to the actual common-mode current flowing in the network cable of the equipment under test (EUT). 3) For the common-mode EUT, the shunt-type AAN with a low LCL of less than about 42 dB yields incorrect measurement results with an error greater than 0.5 dB. 4). A more crucial problem is that, for the differential-mode EUT, the measured disturbance voltage decreases as the EUT common-mode impedance decreases.
We experienced a rare case of tubulointerstitial angiocentric granulomatous vasculitis with focal segmental glomerulosclerosis (FSGS) and associated sarcoidosis. Our patient was an 18-year-old man ...who presented with exertional cough and dyspnea. He also had overt proteinuria (3.0 g/24 h), normal renal function (eGFR 95 mL/min/1.73 m 2 ), heart failure, and hypertension. He had no previous episode of hypertension. These manifestations immediately improved after the administration of antihypertensive therapy that contained an angiotensin-converting enzyme inhibitor, calcium antagonists, beta antagonists, and diuretics. However, he, later on, developed renal dysfunction, with worsening of both proteinuria and hypertension. Renal biopsy was performed and showed epithelioid cells that were arranged concentrically around small blood vessels in tubulointerstitial granulomas. In the glomeruli, the segmental sclerotic lesions were classified as a perihilar variant of FSGS. There were no inflammatory changes, such as a mesangial lesion, inflammatory cell infiltration, fibrinoid necrosis, or crescent formation, and no glomerular granuloma. In the tubulointerstitial granulomas, the intimal elastic lamina of the interlobular arteries was reduplicated, and the intimal wall thickness of renal arterioles was remarkable. After receiving oral prednisolone therapy, the overt proteinuria resolved, the eGFR recovered from 39.4 to 60.6 mL/min/1.73 m 2 , and hypertension was managed more easily. Thereafter, he did not experience any recurrence. The concurrent improvement of renal function and proteinuria by steroid treatment suggested a relationship between the glomerular lesions and the tubulointerstitial granulomatous vasculitis with associated sarcoidosis.
We describe a woman with a 13‐year history of postural instability, vertical gaze palsy and dopa‐responsive parkinsonism ‐ a clinical profile that corresponds to progressive supranuclear palsy (PSP) ...and Parkinson's disease (PD). The patient died at the age of 82 years. Neuropathological features included neuronal loss and gliosis in the substantia nigra, locus ceruleus, dorsal motor nucleus of the vagus, thoracic intermediolateral nucleus and nucleus basalis of Meynert, in addition to the typical pathology of PSP. Immunohistochemical studies demonstrated that PSP‐tau pathology was localized in the central nervous system, but Lewy body‐related α‐synucleinopathy was extensive in the central and peripheral nervous systems. Although PSP and PD may represent independent processes, this case could provide insight into a common defect in either protein phosphorylation or the proteinase surveillance system that contributes to human aging.
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