scholarly journals A Case of Lipoprotein Glomerulopathy with apoE Chicago and apoE (Glu3Lys) Treated with Fenofibrate

2017 ◽  
Vol 7 (2) ◽  
pp. 112-120 ◽  
Author(s):  
Hitoshi Kodera ◽  
Yasuhide Mizutani ◽  
Satoshi Sugiyama ◽  
Toshio Miyata ◽  
Takashi Ehara ◽  
...  

Lipoprotein glomerulopathy (LPG) is characterized by the accumulation of lipoprotein thrombi within glomerular capillaries. This rare disorder is associated with various types of mutations in the apolipoprotein E gene (apoE). Herein, we present a case of LPG with a combination of apoE Chicago (Arg147Pro) and apoE (Glu3Lys) mutations. A 51-year-old Japanese woman presented with severe (3+) proteinuria. The initial renal biopsy showed glomerular capillary dilation and occlusion with lipid granules, a specific characteristic of LPG. Phenotype, genotype, and apoE DNA sequence analyses detected 2 mutations as described above within the same allele. Although both mutations had already been reported in 1 case of LPG each, this is the first time that the combination of the 2 mutations was identified in the same case. Familial analysis detected the same mutations in the patient’s mother. However, she has not suffered LPG thus far. In addition, a re-analysis of the previous LPG case with apoE (Glu3Lys) also identified the apoE Chicago mutation, as was observed in our case. Treatment with fenofibrate and irbesartan was initiated, and urinary protein excretion ceased within 1 year; recurrence was not observed after an additional 2 years of follow-up. A second biopsy after 2 years showed great improvement, with lipoprotein thrombi identified only in 2 of 18 glomeruli.

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
Takashi Suzuki ◽  
Satoru Nakayama ◽  
Haruka Muto ◽  
Shinichi Shimizu ◽  
Yoshiro Otsuki ◽  
...  

Here, we report an extremely rare case of trichoepithelioma (TE)—a benign epithelial tumor originating from the outer root sheath of a hair follicle—arising in an ovarian mature cystic teratoma (MCT) with fluorodeoxyglucose-positron emission tomography (FDG-PET) findings. A 48-year-old Japanese woman presented to our hospital for her annual follow-up of adenomyosis. Ultrasonography and magnetic resonance imaging revealed a left ovarian tumor with irregular-shaped septum, which was suspicious of malignancy. However, tumor marker levels were within normal range. On FDG-PET, the maximum standardized uptake value (SUVmax) of the tumor was 2.9. Laparotomy with left salpingooophorectomy was performed. Pathologic examination revealed the probability of TE, rather than basal cell carcinoma (BCC), arising in an ovarian MCT. After five years of follow-up, the patient had no sign of recurrence. The FDG-PET SUVmax was low in TE, as with other benign tumor. However, future investigation is needed to evaluate the findings of FDG-PET imaging in TE cases.


2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Reiko Suzaki ◽  
Takeaki Shioda ◽  
Izumi Konohana ◽  
Sumiko Ishizaki ◽  
Mizuki Sawada ◽  
...  

Eccrine porocarcinoma is a rare cutaneous neoplasm that mainly affects elderly people and grows slowly over a long period of time but often experiences an accelerated growth phase. Eccrine porocarcinoma may arise de novo or evolve from a pre-existing benign eccrine poroma. We reported a 86-year-old Japanese woman presenting with two reddish-colored pendulated lesions on a keratotic light brown plaque on the right thigh. Dermoscopic examination of the light-brown plaque demonstrated many whitish globular structures in a light-brown background. At the two reddish-colored pendulated lesions, polymorphous and prominent vessel proliferation was observed together with irregularly shaped whitish negative network. Immunohistochemical study demonstrated a positive CEA staining at ductal structures and atypical clear cells of reddish nodules. A diagnosis of eccrine porocarcinoma arising in a pigmented hidroacanthoma simplex was eventually established, and the dermoscopic features of eccrine porocarcinoma from hidroacanthoma simplex was described for the first time.


2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Shiho Iwagaitsu ◽  
Taio Naniwa

A 19-year-old Japanese woman with active Takayasu arteritis despite multiple conventional immunosuppressive therapies with glucocorticoids in combination with intravenous cyclophosphamide, azathioprine, or infliximab with methotrexate and tacrolimus was successfully treated by switching from infliximab to intravenous tocilizumab. Worsening of claudication of the legs and elevated acute phase reactants, including plasma pentraxin-3 levels, were observed during combination therapy with infliximab. Computed tomography demonstrated increased wall thickening with contrast enhancement in the preexisting lesion of the descending aorta and the femoral arteries. After switching from infliximab to tocilizumab, plasma pentraxin-3 levels gradually decreased to the normal range in parallel with the improvement of claudication. Follow-up computed tomographic scans confirmed the marked improvement of these arterial lesions. Moreover, plasma pentraxin-3 level was increased in response to the worsening of claudication that occurred just after switching to a subcutaneous tocilizumab injection. Measurements of plasma pentraxin-3 might be useful for evaluation of the vascular wall inflammation and therapeutic efficacy even during biologic therapy targeting tumor necrosis factor α and interleukin-6.


2013 ◽  
Vol 33 (suppl_1) ◽  
Author(s):  
Hagai Tavori ◽  
Shilin Yang ◽  
Agnes B Fogo ◽  
Daping Fan ◽  
Sergio Fazio

Renal disease is usually associated with lipoprotein abnormalities. Lipoprotein glomerulopathy (LPG) is a unique disease characterized by intraglomerular lipoprotein thrombi, often in the absence of dyslipidemia. Macrophages are not involved in lipid accumulation in LPG. ApoESendai is an apoE variant known to cause LPG in heterozygous carriers. It carries the same charge as the apoE2 common variant, but is due to a proline-for-arginine substitution at position 145 in the apoE3 sequence. The effects of macrophage expression of apoESendai on LPG and atherosclerosis have not been studied.We have characterized renal and arterial responses in apoE-/- or apoE-/-/LDLR-/- (DKO) mice expressing apoESendai exclusively in macrophages. Bone marrow from apoE-/- mice was transduced with lentiviral constructs expressing either human apoE2 or apoESendai, and then transplanted into apoE-/- or DKO recipient mice.Compared to BMT controls, both apoESendai and apoE2 macrophages significantly decreased plasma cholesterol levels in apoE-/- recipients (22.6% and 19.4%, respectively). No changes in serum cholesterol levels were found in DKO recipients compared to controls. Macrophage expression of apoESendai and apoE2 was associated with plasma human apoE levels of 6.9±1.9 and 5.4±1.7 μg/ml, respectively, in apoE-/- recipients, and 18.9±2.8 and 18.4±3.8 μg/ml, respectively, in DKO recipients. All mice displayed extensive kidney tubular lesions, which correlated with the severity of dyslipidemia. While none of the apoE-/- recipients showed any additional kidney damage, DKO mice expressing apoESendai showed clear features of LPG, with lesions in the glomerular capillaries characterized by mesangial cell expansion. Macrophage apoE2 expression by apoE-/- BMT recipients reduced aortic lesion size by 22.7% compare to BMT controls (p<0.01). Surprisingly, macrophage apoESendai expression further reduced aortic lesion size by 27.6% compared to apoE2 expression in apoE-/- mice (p<0.01). A similar 26.1% reduction was seen in DKO recipients transplanted with apoESendai compared to apoE2 expressing macrophages.Our results show for the first time that macrophage expression of apoESendai protects against atherosclerosis while causing glomerular disease.


2020 ◽  
Vol 28 (6) ◽  
pp. 683-687
Author(s):  
Waka Iwashita ◽  
Atsushi Kurabayashi ◽  
Chiharu Tanaka ◽  
Seiji Naganuma ◽  
Takanori Kawamura ◽  
...  

Superficial angiomyxoma is a rare, benign, multilobulated cutaneous tumor composed of stellate and spindle cells, a prominent myxoid matrix, and numerous blood vessels. Superficial angiomyxoma may be indistinguishable from cutaneous lesions of the Carney complex, although superficial angiomyxoma can occur independently of the complex. In this article, we present the case of a 39-year-old Japanese woman with a 40 × 30 mm, focally ulcerated, polypoid superficial angiomyxoma on the left nipple without any evidence of Carney complex. The development of superficial angiomyxoma on the nipples in a patient without the Carney complex is extremely rare. Indeed, only 3 cases of superficial angiomyxoma arising on the nipple have been reported to date, and this is the first such report in Japan. In such cases, the majority of superficial angiomyxoma of the nipples develop in premenopausal women. The possibility of superficial angiomyxoma should be considered for all polypoid nipple lesion, particularly in premenopausal women, and complete excision with follow-up observation should be performed.


2015 ◽  
Vol 6 (1) ◽  
pp. 127-131 ◽  
Author(s):  
Atsuko Sato ◽  
Nami Senda ◽  
Emi Fukui ◽  
Kouichi Ohta

Purpose: To report the presence of retinal angiomatous proliferation (RAP) in an eye with cuticular drusen detected by fluorescein and indocyanine green angiography by confocal scanning laser ophthalmoscopy and by spectral-domain optical coherence tomography (SD-OCT). Methods: Case report of a 65-year-old Japanese woman with cuticular drusen. Results: At her first ophthalmic examination, her visual acuity was 20/20 in both eyes. An ophthalmoscopy showed many small subretinal pigment epithelial deposits in both eyes. These deposits had a ‘saw-tooth pattern' in the SD-OCT images. During the follow-up examination, retinal hemorrhages were observed, and fluorescein angiography showed a ‘stars-in-the-sky' appearance and intraretinal neovascularization. The patient was diagnosed with cuticular drusen associated with RAP. Conclusion: We suggest that the cuticular drusen were associated with RAP, so periodic follow-up examinations are needed for patients with cuticular drusen for the early detection and treatment of RAP.


Crisis ◽  
2016 ◽  
Vol 37 (2) ◽  
pp. 130-139 ◽  
Author(s):  
Danica W. Y. Liu ◽  
A. Kate Fairweather-Schmidt ◽  
Richard Burns ◽  
Rachel M. Roberts ◽  
Kaarin J. Anstey

Abstract. Background: Little is known about the role of resilience in the likelihood of suicidal ideation (SI) over time. Aims: We examined the association between resilience and SI in a young-adult cohort over 4 years. Our objectives were to determine whether resilience was associated with SI at follow-up or, conversely, whether SI was associated with lowered resilience at follow-up. Method: Participants were selected from the Personality and Total Health (PATH) Through Life Project from Canberra and Queanbeyan, Australia, aged 28–32 years at the first time point and 32–36 at the second. Multinomial, linear, and binary regression analyses explored the association between resilience and SI over two time points. Models were adjusted for suicidality risk factors. Results: While unadjusted analyses identified associations between resilience and SI, these effects were fully explained by the inclusion of other suicidality risk factors. Conclusion: Despite strong cross-sectional associations, resilience and SI appear to be unrelated in a longitudinal context, once risk/resilience factors are controlled for. As independent indicators of psychological well-being, suicidality and resilience are essential if current status is to be captured. However, the addition of other factors (e.g., support, mastery) makes this association tenuous. Consequently, resilience per se may not be protective of SI.


2011 ◽  
pp. 100-104
Author(s):  
Thi Thu Nguyen ◽  
Viet Hien Vo ◽  
Thi Em Do

The study use intralesional triamcinolone acetonide injection proceduce for chalazion treatment.1. Objectives: To evaluate results of intralesional triamcinolone acetonide injection for chalazion treatment. 2. Method: This noncomparative prospective interventional trial included 72 chalazions of 61 patients. 3. Results: 61 patients (72 chalazions) with 19 males (31.1%) và 42 females (68.9%), the mean age was 24 ± 9,78 years. 31.1% patients was the first time chalazion and 68.9% patients was more than one times chalazion including 78.6% patients was recurrent at the first position and 21.4% patients occur at new position. 72 chalazions with 16 (22.2%) chalazions was treated before and 56 (77.8%) chalazions wasn’t done that. 72 chalazions with 49 chalazions (68.1%) are local in upper eyelid and 23 chalazions (31.9%) are local in lower eyelid. The mean of chalazion diameter is 6.99 ± 3.03mm. Intralesional triamcinolone acetonide is injected to treat 72 chalazions with 16 (22.2%) chalazions are injected through the route of skin and 56 (77.8%) chalazions are injected through the route of conjunctiva. After 2 weeks follow-up, the success rate was 93.1% and 6.9% failed. 4. Conclusion: intralesional triamcinolone acetonide injection for chalazion treatment is really effective. Key words: chalazion, intralesional triamcinolone acetonide.


2012 ◽  
Vol 2 (1) ◽  
pp. 16
Author(s):  
Takeshi Kusunoki ◽  
Katsuhisa Ikeda

We report a case of a neuroendocrine carcinoma arising in a wound of the postoperative maxillary sinus that was difficult to distinguish from a postoperative maxillary cyst. The patient was a 65-year-old Japanese woman who complained of left exophthalmos with cheek swelling and eye movement disorders. In past history, she had, 40 years previously undergone operation on the bilateral maxillary sinus by Caldwell-Luc’s method. In a preoperative computed tomography, a mass occupied the left maxillary sinus showing irregular densities with destruction of the posterior bone walls and invasion into the left orbital. Both TI and T2 weighted magnetic resonance imaging showed low intensities and unevenness in the mass. We performed a biopsy of the maxillary tumor according to Caldwell-Luc’s method. Histological examination diagnosed neuroendocrine carcinoma. Radiation therapy (total 66Gy) resulted in partial response for this tumor. However, sinonasal neuroendocrine carcinoma has been identified as highly aggressive, with a high probability of recurrence and metastasis.


1990 ◽  
Vol 4 (2) ◽  
pp. 122-128 ◽  
Author(s):  
Yasushi Koitabashi ◽  
Masaaki Ikoma ◽  
Tsuneko Miyahira ◽  
Ryojiro Fujita ◽  
Hitoshi Mio ◽  
...  

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