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Lysosomes are intracellular organelles responsible for degrading diverse macromolecules delivered from several pathways, such as the endo-lysosomal and autophagic pathways. Recent reports have suggested that lysosomes are essential in regulating neural stem cells in developing, adult, and aged brains. However, the activity of these lysosomes has not yet been monitored in these brain tissues. Here, we report a new probe to measure lysosomal protein degradation in brain tissue by immunostaining. Our results demonstrate the fluctuation of lysosomal protein degradation in neural stem cells depending on age and brain disorder. Neural stem cells increase lysosomal activity during hippocampal development in the dentate gyrus, but aging and aging-related disease reduces their activity. In addition, physical exercise increases lysosomal activity in neural stem cells and astrocytes. We hypothesize three different stages of lysosomal activity: the increase in development, the stable state for the adult stage, and the reduction by damages with age or disease.

Background: There are few reports of cases with vertical subluxation. We report our experience of a vertical subluxation of the atlantoaxial joint caused by infection. Case presentation: A 63-year-old man had a 5-month history of neck pain. He was treated for rheumatoid arthritis by a local doctor. At admission to our hospital, he had neck pain and complained of dysphagia and hoarseness. His white blood cell count and C-reactive protein were 7200/mm3 and 4.86 mg/dL. Cervical X-ray showed atlantoaxial subluxation. Computed tomography showed osteolysis around the odontoid process, including the atlantooccipital and atlantoaxial joints, causing vertical subluxation. Magnetic resonance imaging showed spinal cord and brainstem compression and the presence of fluid. Gadolinium-enhanced magnetic resonance imaging showed enhancement around the odontoid process. We performed occipital-cervical spine fixation and C1 laminectomy. Following surgery, his symptoms completely resolved. Bone fusion was confirmed 6 months after surgery. Conclusion: If subluxation of the upper cervical spine occurs rapidly, infection should be considered.

Tooth ankylosis is a pathological condition of periodontal ligament (PDL) restoration after tooth replantation. Platelet-derived growth factor-BB (PDGF-BB) has been proposed as a promising factor for preventing tooth ankylosis. Using rat tooth replantation model, we investigated whether PDGF-BB accelerates the repair of PDL after tooth replantation without ankylosis, and its molecular mechanisms. In PDGF-BB pretreated replanted teeth (PDGF-BB group), ankylosis was markedly reduced and functionally organized PDL collagen fibers were restored; the mechanical strength of the healing PDL was restored to an average of 76% of that in non-replanted normal teeth at 21 days. The numbers of PDGF-Rβ- and BrdU-positive cells in the periodontal tissues of the PDGF-BB group were greater than those of atelocollagen pretreated replanted teeth (AC group). Moreover, in the PDGF-BB group, the periodontal tissues had fewer osteocalcin-positive cells and decreased number of nuclear β-catenin-positive cells compared to those in the AC group. In vitro analyses showed that PDGF-BB increased the proliferation and migration of human periodontal fibroblasts. PDGF-BB downregulated mRNA expressions of RUNX2 and ALP, and inhibited upregulatory effects of Wnt3a on β-catenin, AXIN2, RUNX2, COL1A1, and ALP mRNA expressions. These findings indicate that in tooth replantation, topical PDGF-BB treatment enhances cell proliferation and migration, and inhibits canonical Wnt signaling activation in bone-tooth ankylosis, leading to occlusal loading of the PDL tissues and subsequent functional restoration of the healing PDL. This suggests a possible clinical application of PDGF-BB to reduce ankylosis after tooth replantation and promote proper regeneration of PDL.

The pharmacokinetic-pharmacodynamic (PK-PD) breakpoints (BPs) of garenoxacin (GRNX) and other oral quinolones were calculated using Monte Carlo simulation (MCS) based on the distribution of changes in their plasma concentrations. PK-PD BPs of 400 mg once a day (QD) of GRNX for the free area under the curve/minimum inhibitory concentration (fAUC/MIC) for 30 strains of Streptococcus pneumoniae and 100 strains of gram-negative bacteria (G [－]) were 0.5 and 0.125 μg/mL, respectively. PK-PD BPs of other quinolones for S. pneumoniae/G (－) were 1/0.25 μg/mL for levofloxacin (LVFX) 500 mg QD, 0.5/0.125 μg/mL for moxifloxacin (MFLX) 400 mg QD, 0.0625/0.0156 μg/mL for sitafloxacin (STFX) 50 mg twice a day (BID) (100 mg QD), and 0.125/0.0313 μg/mL for STFX 100 mg BID. We also investigated the hypothetical probability of target attainments (PTAs) of fAUC/MIC for community-acquired pneumonia (CAP) using MCS, in consideration of the isolation frequencies of the three main causative pathogens of CAP: S. pneumoniae, Haemophilus influenzae, and Moraxella catarrhalis. For hypothetical CAP in adults, PTA of fAUC/MIC was 100% with GRNX and MFLX, 96%-97% with STFX at 100 mg BID, 45%-46% with LVFX, and 53%-58% with STFX at 100 mg QD and 50 mg BID. Based on the PK-PD BP, GRNX showed higher fAUC/MIC than the other quinolones tested against the three main pathogens of respiratory infections.

The patella is a rare site for the development of primary tumors. This is the case report of a giant cell tumor (GCT) occurring in the patella in a 25-year-old woman. The patient presented with a 1-year history of occasional right anterior knee pain. The radiological characteristics suggested a benign condition. The intraoperative pathological diagnosis was GCT of the bone. The lesion was treated by radical curettage with adjuvant therapy comprising phenol and ethanol and injection of calcium phosphate cement. Histologically, the tumor consisted of round or spindle-shaped mononuclear cells admixed with numerous osteoclastic giant cells. The patient was asymptomatic and there was no evidence of local recurrence or distant metastasis 16 months after surgery. Although rare, patellar GCT may be included in the differential diagnosis of anterior knee pain and/or swelling, particularly in young adults.

T-2307, an arylamidine compound, has been previously reported to have broad-spectrum in vitro and in vivo antifungal activities against clinically significant pathogens, including Candida species, Cryptococcus neoformans , and Aspergillus species, and is now undergoing clinical trials. Here we investigated the mechanism of action of T-2307 using yeast cells and mitochondria isolated from yeast and rat liver. Nonfermentative growth of Candida albicans and Saccharomyces cerevisiae in glycerol medium, in which yeasts relied on mitochondrial respiratory function, was inhibited at 0.001 to 0.002 μg/ml (0.002 to 0.004 μM) of T-2307. However, fermentative growth in dextrose medium was not inhibited by T-2307. Microscopic examination using Mitotracker fluorescent dye, a cell-permeant mitochondrion-specific probe, demonstrated that T-2307 impaired the mitochondrial function of C. albicans and S. cerevisiae at concentrations near the MIC in glycerol medium. T-2307 collapsed the mitochondrial membrane potential in mitochondria isolated from S. cerevisiae at 20 μM. On the other hand, in isolated rat liver mitochondria, T-2307 did not have any effect on the mitochondrial membrane potential at 10 mM. Moreover, T-2307 had little inhibitory and stimulatory effect on mitochondrial respiration in rat liver mitochondria. In conclusion, T-2307 selectively disrupted yeast mitochondrial function, and it was also demonstrated that the fungal mitochondrion is an attractive antifungal target.

Aims Exposure to glucose and its metabolites in peritoneal dialysis fluid (PDF) results in structural alterations of the peritoneal membrane. Icodextrin-containing PDF eliminates glucose and reduces deterioration of peritoneal membrane function, but direct effects of icodextrin molecules on peritoneal mesothelial cells have yet to be elucidated. We compared the impacts of icodextrin itself with those of glucose under PDF-free conditions on wound healing processes of injured mesothelial cell monolayers, focusing on integrin-mediated cell adhesion mechanisms. Main methods Regeneration processes of the peritoneal mesothelial cell monolayer were investigated employing an in vitro wound healing assay of cultured rat peritoneal mesothelial cells treated with icodextrin powder- or glucose-dissolved culture medium without PDF, as well as icodextrin- or glucose-containing PDF. The effects of icodextrin on integrin-mediated cell adhesions were examined by immunocytochemistry and Western blotting against focal adhesion kinase (FAK). Key findings Cell migration over fibronectin was inhibited in conventional glucose-containing PDF, while icodextrin-containing PDF exerted no significant inhibitory effects. Culture medium containing 1.5% glucose without PDF also inhibited wound healing of mesothelial cells, while 7.5% icodextrin-dissolved culture medium without PDF had no inhibitory effects. Glucose suppressed cell motility by inhibiting tyrosine phosphorylation of FAK, formation of focal adhesions, and cell spreading, while icodextrin had no effects on any of these mesothelial cell functions. Significance Our results demonstrate icodextrin to have no adverse effects on wound healing processes of peritoneal mesothelial cells. Preservation of integrin-mediated cell adhesion might be one of the molecular mechanisms accounting for the superior biocompatibility of icodextrin-containing PDF.

Underwater light environment and photosynthetic accessory pigments were investigated in Ariake Bay in order to understand how change of the pigments occurs in response to the tidal-induced changes in underwater light conditions. We hypothesize that phytoplankton increases photo-protective pigments and decreases light-harvesting pigments under higher light condition in the mixed layer caused by tidal cycle. Contribution rates of non-phytoplankton particles (a nph (400‐700)) for light attenuation coefficient (K d ) was highest (32‐85%), and those of phytoplankton particles (a ph (400‐700)), dissolved organic matter (a g (400‐700)) and water were 6‐32, 6‐21 and 5‐23%, respectively. Mean K d was higher during the spring tide (0.55 ± 0.23 m ‐1 ) than the neap tide (0.44 ± 0.16 m ‐1 ), and the K d difference was caused by the substances resuspension due to the tidal current. In contrast, ratios of photo-protective pigments (diadinoxanthin and diatoxanthin) per chlorophyll a ((DD+DT)/Chl a) were higher during the neap tide (0.10 ± 0.03 mg mg-Chl a ‐1 ) than the spring tide (0.08 ± 0.03 mg mg-Chl a ‐1 ). And there was significant positive correlation between (DD+DT)/ Chl a and mean relative PAR in the mixed layer ( Imix ). Moreover, there was significant negative correlation between ratios of light-harvesting pigments (fucoxanthin) per Chl a (Fuco/Chl a) and Imix . These results suggested that phytoplankton in Ariake Bay increase photo-protective pigments and decrease light-harvesting pigments in the higher light condition of less turbid, shallower mixed layer during neap tide than spring tide.

Objectives: T-2307, a novel arylamidine synthesized at Toyama Chemical Co., Ltd, has in vitro and in vivo broadspectrum activities against pathogenic fungi. T-2307 particularly exhibits potent in vitro and in vivo activity against Candida albicans, suggesting that its uptake might be mediated by a transport system. In this report, we studied the uptake of T-2307 in C. albicans. Methods: C. albicans cells and rat hepatocytes were exposed to 0.02 m M[ 14 C]T-2307. After incubation, the reaction mixture was concentrated and layered on a silicon layer (mixture of silicon oil and liquid paraffin) inside a tube. The tube was then centrifuged to transfer cells into the bottom layer (sodium hydroxide) for solubilization. The bottom layer was neutralized and measured for radioactivity. Results: T-2307 was concentrated from the extracellular medium by C. albicans cells in 10 mM phosphate buffer solution supplemented with 1% glucose by 3200- to 5100-fold. The accumulation was approximately two orders of magnitude greater than that achieved with a rat hepatocyte preparation. T-2307 uptake was sensitive to temperature and extracellular pH, and was reduced in the presence of inhibitors of mitochondrial respiration, oxidative phosphorylation and plasma membrane proton pump, and by an uncoupler. Furthermore, T-2307 uptake was concentration dependent and an Eadie‐Hofstee plot suggested the involvement of two transport systems. Conclusions: The considerably higher concentrations of T-2307 were selectively accumulated in C. albicans via transporter-mediated systems, as compared with the concentrations in rat hepatocytes. This transportermediated uptake of T-2307 contributes to its potent anticandidal activity.

BACKGROUND: Bioincompatible peritoneal dialysis fluids (PDFs) cause pathological changes in the peritoneal membrane, related to membrane dysfunction and progressive peritoneal fibrosis. We investigated the effects of Pro-His-Ser-Arg-Asn (PHSRN) peptide, one of the fibronectin cell-binding domains that activates integrins and reinforces wound healing, on peritoneal remodelling in a rat peritoneal injury model undergoing peritoneal dialysis. METHODS: The peritoneal mesothelial monolayer was removed by a stripping procedure in rats receiving conventional high glucose-containing PDF supplemented with or without PHSRN or control His-Ser-Pro-Asn-Hrg (HSPNR) peptides. Effects of PHSRN on cell motility and signalling molecules were examined in cultured rat peritoneal mesothelial cells (RPMCs) and normal rat kidney fibroblasts (NRKs). RESULTS: The cytokeratin- and HBME-1-positive mesothelial cell monolayer was selectively removed by the procedure. By day 6, HBME-1-positive cells had regenerated to 53.3 +/- 6.5% of the peritoneal surface in the control group. Regeneration of the mesothelial layer was delayed in the PDF group (35.2 +/- 10.2%, P < 0.05), but PHSRN reversed the effects of PDF (51.7 +/- 9.6%, P < 0.05). PDF treatment increased thickening of granulomatous submesothelial tissue and numbers of ED1-, CD31- and alpha-smooth muscle actin-positive cells, but PHSRN ameliorated these effects. HSPNR had no effects on mesothelial regeneration or peritoneal wound healing. PHSRN, but not HSPNR, recovered glucose-induced inhibition of cell motility and phosphorylation of focal adhesion kinase and its downstream p130(Cas) in RPMCs and NRKs. CONCLUSIONS: These results suggest that PHSRN has beneficial effects on peritoneal regeneration by reducing the inhibitory effects of conventional PDF on integrin-mediated wound healing.

Aims Insulin-like growth factor (IGF)-1 is a major mitogenic growth factor for mesangial cells (MCs). Statins slow the progression of chronic kidney disease by affecting inflammatory cell signaling pathways, in addition to improving lipid profile, however, no studies have investigated the effects of fluvastatin on mitogen-activated protein (MAP) kinase activity or MC proliferation in kidney cells. We investigated the effects of fluvastatin on IGF-1-induced activation of intracellular signal pathways and MC proliferation, and examined the inhibitory mechanisms of fluvastatin. Main methods Western blotting and cell proliferation assay were used. Key findings IGF-1 induced phosphorylation of extracellular-related kinase (ERK)1/2, MAP or ERK kinase (MEK)1/2, and Akt, expression of cyclin D1, and MC proliferation in cultured human MCs. Fluvastatin or PD98059, an MEK1 inhibitor, completely abolished IGF-1-induced MEK1/2 and ERK1/2 phosphorylation and MC proliferation, whereas inhibition of Akt had no effect on MC proliferation. Mevalonic acid prevented fluvastatin inhibition of IGF-1-induced MEK1/2 and ERK1/2 phosphorylation, cyclin D1 expression, and MC proliferation. Significance Fluvastatin inhibits IGF-1-induced activation of the MAP kinase pathway and MC proliferation by mevalonic acid depletion, and might have renoprotective effects by inhibiting IGF-1-mediated MC proliferation.

We propose Symbiosis Communicator for Advanced and Preserved Environment system, SCAPE System, which consists of virtual, intelligent, and Kansei media. This paper shows the analysis and model about a mechanism that makes different media information harmonized. We use building data as 3D information and urban landscape image as 2D information to retrieve different kinds of multimedia data information. We construct a system that 3D building data harmonized with a urban landscape are retrieved when the urban landscape image is put as a key. As a result, people who are not specialist can tell own opinions to specialists and they can take part in urban planning.

Activating receptor activator of NF-kappaB (RANK) and TNF receptor (TNFR) promote osteoclast differentiation. A critical ligand contact site on the TNFR is partly conserved in RANK. Surface plasmon resonance studies showed that a peptide (WP9QY) that mimics this TNFR contact site and inhibits TNF-alpha-induced activity bound to RANK ligand (RANKL). Changing a single residue predicted to play an important role in the interaction reduced the binding significantly. WP9QY, but not the altered control peptide, inhibited the RANKL-induced activation of RANK-dependent signaling in RAW 264.7 cells but had no effect on M-CSF-induced activation of some of the same signaling events. WP9QY but not the control peptide also prevented RANKL-induced bone resorption and osteoclastogenesis, even when TNFRs were absent or blocked. In vivo, where both RANKL and TNF-alpha promote osteoclastogenesis, osteoclast activity, and bone loss, WP9QY prevented the increased osteoclastogenesis and bone loss induced in mice by ovariectomy or low dietary calcium, in the latter case in both wild-type and TNFR double-knockout mice. These results suggest that a peptide that mimics a TNFR ligand contact site blocks bone resorption by interfering with recruitment and activation of osteoclasts by both RANKL and TNF.

S100A4 is an intracellular calcium-binding protein expressed by osteoblastic cells. However, its roles in bone physiology are unknown. Because before matrix mineralization, its expression is markedly diminished, we hypothesized that S100A4 negatively regulates the mineralization process. In this study, we investigated the effects of the inhibition of S100A4 synthesis on osteoblast differentiation and in vitro mineralized nodule formation. Inhibition of S100A4 synthesis was achieved by an antisense approach in the mouse osteoblastic cell line MC3T3-E1. Cell clones that synthesized low levels of S100A4 (AS clones) produced markedly increased number of mineralized nodules at much earlier stages in comparison with controls as demonstrated by Alizarin red S and von Kossa staining. The expression of type I collagen (COLI) and osteopontin (OPN) increased in AS clones compared with controls. Bone sialoprotein (BSP) and osteocalcin (OCN), molecules associated with mineralization and markers for mature osteoblastic phenotype, were expressed in AS clones before their detection in controls. Because S100A4 was not localized in the nucleus of MC3T3-E1 cells and AS clones, it is unlikely that S100A4 directly regulates the expression of these genes. Moreover, the expression of Cbfal/Osf-2 and Osx, transcription factors necessary for the expression of osteoblast-associated genes, remained unchanged in AS clones, indicating that S100A4 may be downstream to these transcription factors. These findings indicate that S100A4 is a novel negative regulator of matrix mineralization likely by modulating the process of osteoblast differentiation.

It has hitherto been difficult to treat landscape images in a database, since their physical characteristics have been described empirically. This paper analyzes the correlation between the physical characteristics (colors, including their brightness, directions, and positions) and their associated adjectives, so that a landscape image can be modeled by using the adjectives. As a result, landscape images can be evaluated automatically, and an unknown landscape image can be retrieved and evaluated. Experiments have been carried out to show the usefulness of the proposed method. © 2001 Scripta Technica, Syst Comp Jpn, 32(6): 62–71, 2001

Not only facial expressions but also body gestures and postures play an important role in non-verbal communication. Whilst evidence shows that affective standing postures account for at least four affective dimensions - arousal, valence, potency and avoidance - it is not clear if the same is true for sitting postures. We investigated the perception of sitting postures and the body descriptors that guide these perceptual processes. Japanese body postures were collected and measured through conventional sensors. Japanese observers were recruited for the study. The results show that, for Japanese observers, the three dimensions of arousal, valence and dominance were necessary to account for most of the variance in the perception of the set of affective postures. It also showed that, for Japanese observers, the rating of dominance was modulated by the position of the legs, arms and trunk. We build an emotion estimation system in real time and evaluate the system.

Mizoribine (MZR) has shown to be effective against antineutrophil cytoplasmic antibody (ANCA)-related vasculitis; however, no reports have described the successful treatment of steroid-resistant ANCA-related vasculitis with MZR in patients with renal insufficiency requiring hemodialysis. We herein report the case of a 39-year-old man undergoing hemodialysis in whom MZR successfully lowered the myeloperoxidase (MPO)-ANCA titer accompanied by remission of interstitial pneumonia, together with the pharmacokinetics of MZR. The patient developed severe renal insufficiency and interstitial pneumonia, and was started on hemodialysis. Although prednisolone was administered followed by azathioprine, the MPO-ANCA level and interstitial pneumonia showed insufficient improvement. Azathioprine was replaced by MZR and the administered dose of MZR was determined by measuring serum concentrations of MZR at the start of the dialysis session; this was because we confirmed that MZR could only be removed via dialysis, and that the serum concentration of MZR was maintained until the next dialysis session. The maintenance dose was finally set at MZR 75 mg after each dialysis. Subsequently, the ANCA titer decreased and interstitial pneumonia resolved without any MZR-related side effects. This case demonstrates that MZR is safe and effective, even in patients with steroid-resistant ANCA-related vasculitis undergoing hemodialysis, and can be monitored by measuring serum concentrations of MZR.

Not only facial expressions but also body gestures and postures play an important role in non-verbal communication. Whilst evidence shows that affective standing postures account for at least four affective dimensions - arousal, valence, potency and avoidance - it is not clear if the same is true for sitting postures. In addition, whilst there is a large body of work investigating cross-cultural differences in the perception of facial expressions, there is very little work on the cross-cultural perception of body expressions. We investigated the minimal universality and cross-cultural difference in the perception of sitting postures and the body descriptors that guide these perceptual processes. Japanese body postures were collected and measured through conventional sensors. Japanese and British observers were recruited for the cross-cultural study. The results show that, for Japanese observers, the three dimensions of arousal, valence and dominance were necessary to account for most of the variance in the perception of the set of affective postures whilst, for British observers, only valence and arousal were necessary. An analysis of the body descriptors highlighted a few differences in the way these were associated with affective dimensions. It also showed that, for Japanese observers, the rating of dominance was modulated by the position of the legs, arms and trunk.

鋼中非金属介在物の除去や形態制御による鋼の清浄化や機能化は重要な課題であり,介在物粒子の生成過程の解明と起源探索手法の確立が必要である.一方,一次ビームにGa+収束イオンビームを用いたサブミクロン二次イオン質量分析(SIMS)装置は,高い切断加工能力と0.1μm以下の高空間分解能を有し,微小領域並びに数μm程度の微粒子に対する定量分析・三次元元素分布解析に大変有効である.著者らは介在物粒子のキャラクタリゼーション手法の確立を目的として,Ti添加極低炭素鋼中の介在物粒子に対し以下の分析を行った.始めに,電子線マイクロアナリシス法により任意の粒子に対して粒別分析を行い,供試鋼中の介在物が三タイプに分類されることを明らかにした.次いで,各タイプの代表的な粒子に対してサブミクロンSIMS装置を用いて粒別定量分析を行い,粒内元素分布と任意断面の化学組成を得た.以上の分析結果から,供試鋼中に含まれる介在物粒子の生成過程に関する検討を行い重要な知見を得た.

Higher order local autocorrelation (HLAC) features are used in various 2D image applications. Recent three dimensional extensions of HLAC features (3D HLAC) have made possible the analysis of solid textures including solid textures. Typically, 3D HLAC features are computed by a limited number of three dimensional mask patterns, and the order of the mask patterns is limited up to the second order. In this paper, the orders of the mask patterns are increased up to the 26th order. Since there are a large number of combinations of mask patterns, we have implemented a simulation program for finding all their combinations in a systematic manner. Also the various subsets of the 3D HLAC mask patterns are computed by limiting the displacement regions of the masks. The 3D HLAC features were computed as local features rather than global features, and the bag of features (BoF) approach was applied for comparing local features. Our experimental program extracted pattern features from solid textures by using the extended 3D HLAC mask patterns. In our preliminary experiments, databases of solid textures were synthesized from 2D images. A similarity retrieval system for the solid textures has been implemented, and its extension to the 3D HLAC features performed well in retrievals.

Strongyloidiasis, a chronic infection caused by the intestinal parasite Strongyloides stercoralis, is prevalent in the Nansei Islands of Japan. Here, we report our findings on a case of strongyloidiasis complicated with steroid-resistant minimal change nephrotic syndrome in a 69-year-old male resident of Fukuoka Prefecture who had lived in Yakushima, one of the Nansei Islands, until age 15. In October 2006, he developed proteinuria and edema, and was diagnosed with minimal change nephrotic syndrome on the basis of the renal biopsy findings. Following treatment with prednisolone, the level of proteinuria decreased to 0.29 g/day by day 35. However, 5 days later (day 40), the patient developed persistent watery diarrhea and vomiting, leading to dehydration and malnutrition. Pneumonia and bacterial meningitis subsequently developed (day 146); filarial (infectious-type) and rhabditiform (noninfectious-type) S. stercoralis larvae were detected for the first time in the patient’s sputum, gastric juice, feces, and urine. Although treatment with ivermectin was started immediately and the parasitosis responded to the treatment, the patient died of sepsis. Consequently, although strongyloidiasis is a rare infection except in endemic regions, it is essential to consider the possibility of this disease and begin treatment early for patients who have lived in endemic areas and who complain of unexplained diarrhea during steroid-induced or other immunosuppression.

脳腱黄色腫（cerebrotendinous xanthomatosis, CTX）はステロール27位水酸化酵素の障害による脂質代謝異常を呈す，常染色体劣性遺伝の稀な疾患である．今回，我々はCTXの経過中に微小変化型ネフローゼ症候群を合併した症例を経験した．プレドニゾロンの投与のみでは不完全寛解I型までしか改善せず，プレドニゾロンの漸減に伴い再燃したが，LDLアフェレーシスの併用で血清コレスタノール値が低下し完全寛解となった．本例では脂質代謝異常とネフローゼ症候群の発症機序に何らかの関連が疑われた．