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指定難病 — No.47

バージャー病

検索語 Thromboangiitis Obliterans ・ 最終更新 2026-07-21 18:34 ・ 最新に更新

Data Sheet
指定 No.47
Src PubMed · CT.gov · jRCT

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( 01 )EVIDENCE / PUBMED · 5件

世界の論文

直近の研究を、やさしい日本語で

各論文の見出しにある「確からしさ」は、その研究がどれくらい信頼できるかの目安です。「理論段階」はまだ仮説に近く、下にいくほど多くの患者で検証されていて、「メタ解析」がもっとも信頼できます。

ランダム化比較試験(RCT)
MK-01 · PMID 42429384

Systemic Exposure and Multicomponent Pharmacokinetics of Mailuoning Oral Liquid: Integrated Ultra-High-Performance Liquid Chromatography-High-Resolution Mass Spectrometry Profiling and Ultra-High-Performance Liquid Chromatography-Tandem Mass Spectrometry Quantification

Abstract / 原文

Mailuoning oral liquid (MLN) is a state-approved multi-herb formula that has been prescribed for 25 years to clear heat, nourish yin, invigorate blood, and resolve stasis. Although randomized controlled trials and real-world evidence support its efficacy in thromboangiitis obliterans, deep-vein thrombosis, and the convalescent phase of cerebral infarction, a systematic search identified no pharmacokinetic data on its bioactive constituents. This lack of pharmacokinetic data impedes evidence-based dose refinement and international acceptance. Here, we employed ultra-high-performance liquid chromatography with Quadrupole-Exactive Orbitrap high-resolution mass spectrometry (UHPLC-Q-Exactive Orbitrap HRMS) to track 39 MLN-derived compounds in rat plasma, intestinal contents, and 10 tissues; 15 with prominent systemic exposure were designated as key pharmacokinetic markers. A selective and sensitive UHPLC-tandem MS assay was subsequently developed and fully validated for simultaneous quantification of these 15 circulating components. The assay exhibited excellent linearity (r ≥ 0.9974) for all analytes, with precision, accuracy, extraction recovery, and matrix effects meeting standard bioanalytical validation criteria. All compounds demonstrated stability under the tested storage conditions. Following oral administration of MLN at 8.1 and 16.2 mL·kg-1 in rats, all 15 components were rapidly absorbed, reaching peak plasma concentrations within approximately 1 h. Systemic exposure increased in a clear dose-dependent manner, with structurally related compounds showing similar pharmacokinetic profiles. Notably, the iridoids secoxyloganin, harpagide, and secologanic acid yielded the highest AUC0-24 h values, indicating they are the principal systemic exposure markers. This study establishes the first comprehensive pharmacokinetic profile for MLN and provides a robust, validated analytical platform. By creating a crucial link between quantitative systemic exposure and efficacy, our work supplies a scientific foundation for refining dosage regimens, establishing quality specifications, and promoting the evidence-based clinical application of this widely prescribed traditional Chinese medicine formula.

Journal
Journal of separation science(2026 Jul)
Authors
11名
Type
Journal Article
PubMedで原文を見る
観察研究
MK-02 · PMID 42355688

Efficacy of the Tibial Transverse Bone Transport Technique in the Management of Thromboangiitis Obliterans: A Systematic Review

Abstract / 原文

Background: Thromboangiitis obliterans (TAO) is a nonatherosclerotic inflammatory vascular disorder affecting small- and medium-sized vessels, often leading to critical limb ischemia and a high risk of amputation. Conventional medical and surgical treatments remain limited, particularly for advanced diseases. Tibial transverse bone transport (TTT), based on the principles of distraction osteogenesis, has emerged as a novel technique to promote angiogenesis and improve microcirculation. This systematic review evaluated the clinical efficacy and safety of TTT in the management of TAO. Methods: A systematic review was conducted according to the PRISMA guidelines. A comprehensive search of PubMed, Scopus, Web of Science, ScienceDirect, and Google Scholar was performed until December 2025. Eligible studies included clinical investigations that evaluated TTT in patients with TAO. Data on patient characteristics, surgical techniques, clinical outcomes, and complications were extracted and analyzed descriptively because of the heterogeneity in study design and reporting. Results: Ten studies involving 368 patients were included in this review. TTT was consistently associated with significant clinical improvement, including pain relief, increased claudication distance, and ulcer healing, which were typically observed within weeks after the procedure. Limb salvage rates were high, with major amputation rates generally ranging from 3.3% to 13.3%. Objective improvements in perfusion parameters have also been reported in several studies. The most common complication was pin-site infection (up to 30%), while fractures, delayed consolidation, and osteomyelitis were less frequent complications. Conclusions: Current evidence suggests that TTT is a promising limb-salvage strategy for TAO and is associated with favorable clinical and functional outcomes, with an acceptable complication profile. However, the available evidence remains limited, partly because of the rarity of TAO and the specialized nature of the TTT procedure. Most available studies are observational, and further high-quality prospective and randomized trials are required to validate the long-term efficacy of this technique.

Journal
Journal of clinical medicine(2026 Jun)
Authors
2名
Type
Journal Article, Review
PubMedで原文を見る
不明
MK-03 · PMID 42298937

Thromboangiitis Obliterans of the Colon: An Unusual Manifestation of Buerger's Disease

Journal
The Israel Medical Association journal : IMAJ(2026 Jan)
Authors
6名
Type
Journal Article
PubMedで原文を見る
不明
MK-04 · PMID 42297648

Corrigendum to "Integrated metabolomics and network pharmacology study on Si-miao-yong-an Decoction in regulating sphingolipid metabolism and improving vascular injury on thromboangiitis obliterans" [J. Ethnopharmacol. 357 (2026) 120868]

Journal
Journal of ethnopharmacology(2026 Jun)
Authors
7名
Type
Published Erratum
PubMedで原文を見る
観察研究
MK-05 · PMID 42268806

Clinical outcomes of critical limb ischemia in Buerger disease: A contemporary analysis from an academic referral center in Jordan

Abstract / 原文

OBJECTIVE: Buerger disease (BD), thromboangiitis obliterans, is a rare occlusive peripheral arterial disease affecting young smokers. To date, no study has investigated the burden of this disease in Jordan. This study aimed to assess the clinical course and factors associated with adverse outcomes of BD patients with critical limb ischemia (CLI) in Jordan. METHODS: The medical records of all patients with BD who presented with CLI at King Abdullah University Hospital in Jordan between January 2015 and January 2024 were retrospectively retrieved. The epidemiological profile of retrieved cases was collected along with the course of treatment, adverse events, and outcome. Statistical analyses were performed to assess the relationships between patient presentation, treatment course, and outcome. RESULTS: The study comprised 41 BD patients with Rutherford categories (RC) (IV-VI). The majority were males (95.1%) in their third decade (51.2%). Most patients (92.7%) presented with ischemic ulcerations (RC V, VI). A multivariable regression model revealed that increased C-reactive protein levels (OR = 1.044, 95% CI [1.003, 1.087], p = 0.035), gangrene at presentation (RC VI) (OR = 11.545, 95% CI [1.444, 92.332], p = 0.021), and limb infections at admission (OR = 7.745, 95% CI [1.182, 50.755], p = 0.033) were practical predictors of ending up with major limb amputation. CONCLUSION: In Jordan, BD is associated with a high rate of amputation, which imposes considerable financial and social burdens. Prompt recognition, early management, and stringent smoking cessation measures are imperative to slowing disease progression.

Journal
PloS one(2026)
Authors
8名
Type
Journal Article
PubMedで原文を見る
( 02 )TRIALS / JAPAN · 0件

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( 03 )REGISTRY / jRCT

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日本の公式レジストリで全件を確認

上の一覧は ClinicalTrials.gov の一部です。日本国内の治験の多くは、日本の公式レジストリ jRCT にのみ登録されています。下記から最新の全件をご確認ください。

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