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Ohshima K.,National Hospital Organization Okayama Medical Center
Journal of clinical and experimental hematopathology : JCEH | Year: 2013

There are no reports on the effect of anti-allergic agents against IgG4-related disease. We herein report a case of IgG4-related dacryoadenitis that is believed to have regressed due to the administration of anti-allergic agents. A 57-year-old woman consulted us because of bilateral temporal upper eyelid swelling and induration. She had also been suffering from allergic rhinitis and allergic conjunctivitis for 20 years. We performed an incisional biopsy of the lesion. With respect to the pathology, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type was strongly suspected. On obtaining consent from the patient, follow-up alone was to be continued without radiation therapy. In addition to the observation of lacrimal gland lesions, the administration of epinastine hydrochloride at a dosage of 20 mg/day and 0.01% betamethasone eye drops twice a day to both eyes was commenced in order to treat both allergic rhinitis and allergic conjunctivitis. The lacrimal gland lesion decreased in size over time, becoming predominantly normal 7 years after the commencement of agent administration. We therefore re-examined the blood and pathology specimens. As a result, the serum IgG4 level was found to have increased to 540 mg/dl, while IgG4/IgG was 36.2%. The pathological diagnosis was revised to IgG4-related dacryoadenitis. The hypotheses of spontaneous remission and/or the effect of epinastine hydrochloride administration can be proposed regarding the mechanism by which the lacrimal gland lesion decreased in size. [J Clin Exp Hematop 53(1): 53-56, 2013]. Source


Sogabe Y.,Mitoyo General Hospital | Ohshima K.-I.,National Hospital Organization Okayama Medical Center | Azumi A.,Kobe Kaisei Hospital | Takahira M.,Kanazawa University | And 4 more authors.
Graefe's Archive for Clinical and Experimental Ophthalmology | Year: 2014

Background: It is well-known that the lacrimal gland (LG) may be affected in IgG4-related ophthalmic disease (IgG4ROD). Recently, IgG4-related ophthalmic lesions other than those of the lacrimal gland have been reported. However, no study to date has revealed the details of these lesions. This study was conducted to evaluate the location and frequency of lesions found in conjunction with IgG4ROD using radiological imaging. Methods: Radiological images and clinical records of 65 patients collected from seven institutions in Japan were reviewed retrospectively. All patients had been pathologically diagnosed with IgG4ROD. Patients of mucosa-associated lymphoid tissue lymphoma associated with IgG4-related lesions were excluded. Orbital magnetic resonance imaging or computed tomography findings were evaluated. Results: Of the 65 patients, 31 (47.7 %) had lesions involving the LG alone, whereas 34 (52.3 %) had lesions involving the areas other than LG, including eight patients who had lesions without any LG involvement. IgG4-related ophthalmic lesions included LG enlargement in 57 patients (87.7 %), trigeminal nerve branch enlargement in 25 (38.5 %), extraocular muscle enlargement in 16 (24.6 %), diffuse orbital fat lesions in 15 (23.1 %), orbital mass lesions in 11 (16.9 %), eyelid lesions in eight (12.3 %), and nasolacrimal duct lesion in one (1.5 %). Six patients (9.2 %) presented with visual disturbance due to optic nerve disturbance, eight (12.3 %) with a restriction of ocular movement, and 19 (29.2 %) with exophthalmos. Conclusions: Thirty-four (52.3%) of the 65 IgG4ROD patients had lesions in areas other than LG. Lesions were found in the trigeminal nerve branch including pterygopalatine fossa, extraocular muscles, orbital fat, eyelid, and nasolacrimal duct. © Springer-Verlag 2014. Source


Fukuhara T.,National Hospital Organization Okayama Medical Center
PLoS ONE | Year: 2015

Since the launch of the novel medical reimbursement system Diagnosis Procedure Combination (DPC) in 2003 in Japan, inpatient data has been accumulated over time as part of a Japanese governmental nationwide database. This is partially accessible by the public, and this study examined the adequacy of this database as epidemiological research material by extracting the data relating to aneurysmal subarachnoid hemorrhage (aSAH) with special attention given to the limitations that this involves. Datasets after 2010 are considered suitable for analysis because of the numbers of participating hospitals and the analysis term. Extracting the data by prefecture, those with a continuously high aSAH incidence were Aomori, Iwate, Akita, Yamagata, Kochi and Kumamoto Prefectures, and those with low aSAH incidence were Kanagawa, Shiga, Kyoto, Shimane and Ehime Prefectures. Although these obtained results are informative, a publically-accessible DPC database has several limitations. Some limitations have been resolved: the analyzed term each year is now 12-months and the number of participating hospitals seems to have stabilized around 1700. However, other limitations such as masking the numbers in each hospital reporting less than 10 patients still exist, so careful and critical interpretation is necessary in utilizing a publically-accessible DPC database. Considering the potential of this database as material for epidemiological research, future analysis of the entire DPC database by qualified researchers is desirable. © 2015 Toru Fukuhara. Source


Ohshima K.-I.,National Hospital Organization Okayama Medical Center | Sogabe Y.,Section of Ophthalmology | Sato Y.,Okayama University of Science
Japanese Journal of Ophthalmology | Year: 2012

Purpose: To investigate the frequency of infraorbital nerve enlargement (IONE) in orbital lymphoproliferative disorders, and to show that IONE can contribute to the clinical diagnosis of IgG4-related orbital diseases (IgG4- ROD). Subjects and methods: 71 cases in which orbital lymphoproliferative disorders were diagnosed at Okayama Medical Center and Mitoyo General Hospital from April, 2004 to March, 2011 were investigated. The male-to-female ratio was 39:32, and the age range 27-87 years old (average age 64.1 years). Whenever the coronal section of the infraorbital nerve was larger than that of the optic nerve on MRI, it was defined as IONE. Results: The breakdown of the 71 cases was: 45 cases of non-Hodgkin lymphoma, 16 cases of IgG4-ROD, 5 cases of reactive lymphoid hyperplasia, and 5 cases of idiopathic orbital inflammation. Of these, a total of 9 cases had IONE. The incidence of IONE was compared between the IgG4- ROD patient group and the non-IgG4-ROD patient group and was significantly higher in the IgG4-ROD patient group (p < 0.0001). Conclusion: If IONE is observed in a case of orbital lymphoproliferative disorders on MRI, then it is highly possible that such a case is IgG4-ROD. © Japanese Ophthalmological Society 2012. Source


Ogawa A.,National Hospital Organization Okayama Medical Center | Firth A.L.,Salk Institute for Biological Studies | Smith K.A.,University of Illinois at Chicago | Maliakal M.V.,University of Illinois at Chicago | Jason X.-J.,University of Illinois at Chicago
American Journal of Physiology - Cell Physiology | Year: 2012

Plateletderived growth factor (PDGF) and its receptor are known to be substantially elevated in lung tissues and pulmonary arterial smooth muscle cells (PASMC) isolated from patients and animals with pulmonary arterial hypertension. PDGF has been shown to phosphorylate and activate Akt and mammalian target of rapamycin (mTOR) in PASMC. In this study, we investigated the role of PDGF-mediated activation of Akt signaling in the regulation of cytosolic Ca 2+ concentration and cell proliferation. PDGF activated the Akt/mTOR pathway and, subsequently, enhanced store-operated Ca 2+ entry (SOCE) and cell proliferation in human PASMC. Inhibition of Akt attenuated the increase in cytosolic Ca 2+ concentration due to both SOCE and PASMC proliferation. This effect correlated with a significant downregulation of stromal interacting molecule (STIM) and Orai, proposed molecular correlates for SOCE in many cell types. The data from this study present a novel pathway for the regulation of Ca 2+ signaling and PASMC proliferation involving activation of Akt in response to upregulated expression of PDGF. Targeting this pathway may lead to the development of a novel therapeutic option for the treatment of pulmonary arterial hypertension. © 2012 the American Physiological Society. Source

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