Fabry disease (FD) is a rare X chromosome-linked disorder and can be easily misdiagnosed. Here, we report the case of a 69-year-old male patient with FD who developed heart failure and showed extremely high pulmonary artery pressure. His initial symptom was recurrent atrial fibrillation. The left and right atrial inner diameters were large, and the ventricular wall was thick. Gene analysis which showed GLA c.215T>C p.Met72Thr mutation and single photon emission computed tomography indicated the diagnosis of FD with coronary microvascular dysfunction. The patient was prescribed anti-heart failure drugs, including vericiguat. Following the treatment, his heart function and microvascular perfusion significantly improved, which might be due to the beneficial effects of vericiguat.

BACKGROUND: Microcirculatory pathology is one of the pathophysiological theories of migraine, which may present as visually subclinical lesions. Image markers of cerebral small vessel disease (CSVD) have been investigated in elderly migraineurs. However, past studies looked at only part of image features, and the conclusions may have been hindered by confounding factors. The relationship between migraine and CSVD signs needs reliable demonstrations.
METHODS: We conducted a case-control study by recruiting episodic young migraineurs from a tertiary headache centre, with tension-type headache (TTH) and healthy controls. Distinct image features of microvascular damage and baseline characteristics across groups were assessed, and multivariate linear regression was performed to evaluate the risk factors for image abnormalities in migraineurs.
RESULTS: Forty-eight migraineurs, 32 TTHs and 49 healthy controls were included. The median age was 32 year-old. 58.7% of the participants were female. The Scheltens score and volume of white matter hyperintensities (WMHs) in migraineurs, and the number of Virchow-Robin spaces (VRSs) in both migraineurs and TTHs were different from those in normal controls. No lacunar infarct-like lesions (ILLs) or cerebral microbleeds (CMBs) were found. Age, education level (high level: β = -2.23, lobar WMHs), attack duration (long duration: β = 3.81, lobar WMHs) and attack frequency were independent risk factors for Scheltens score and volume of WMH in migraineurs. Migraine aura (β = -2.389), attack frequency and education level were correlated with the number of VRSs.
CONCLUSIONS: Migraine was associated with WMHs and VRSs. Aura, attack duration, attack frequency, age and education level were risk factors for image abnormalities of CVSD in migraineurs.
CONCLUSIONS: This study provides a novel and comprehensive landscape of CSVD MRI features in young migraineurs, and it fills the blank of CMBs and VRSs which received less attention, with more persuasive, more reliable and stronger evidence of the association between CSVD and migraine. Our results also imply some new feature of TTH and the possible pathophysiology of the migraine course as well as new clues for the early management of migraine in terms of visual brain damage.

OBJECTIVE: Was to conduct the analysis of patient\'s clinical observation with acute dinitrophenol poisoning, admitted to a toxicological department of CCH №6 of Izhevsk, Udmurt Republic in 2021 yr. In this clinical case report, a 19 years old girl, who took 20 tablets of dinitrophenol, illegally obtained in online-shop, died. The fatal outcome was realized by the uncoupling of oxidative phosphorylation mechanism and cellular respiration, which in its turn led to serious dystrophic changes in all organs and tissues. Disorders of hemodynamics and blood rheological properties dominated in poisoning pathogenesis, led to congestion, stasis in microcirculatory vessels, hyperpermeability with multiple perivascular hemorrhages in organs, occurrence of piecemeal necrosis in kidneys and liver, nephrosis and nonspecific reactive hepatitis. Production ATP from ADP becomes impossible in these conditions, and respiratory energy chain completely disappears as heat, that explains the heat-increasing and fat-burning effects of dinitrophenol.
UNASSIGNED: Работы — проведение анализа клинического наблюдения пациентки с острым отравлением динитрофенолом, поступившей в токсикологическое отделение БУЗ УР ГКБ №6 Ижевска Удмуртской Республики в 2021 г. В данном клиническом случае наступил летальный исход у 19-летней девушки, принявшей с целью похудения 20 таблеток динитрофенола, незаконно приобретенного в интернет-магазине. Смертельный сценарий реализовался через механизм разобщения окислительного фосфорилирования и клеточного дыхания, который, в свою очередь, привел к серьезным дистрофическим изменениям во всех органах и тканях. В патогенезе отравления превалировали нарушения гемодинамики и реологических свойств крови, приведшие к венозному полнокровию, стазам в сосудах микроциркуляторного русла, повышенной проницаемости сосудистых стенок с множественными периваскулярными геморрагиями в органах, развитием мелкоочаговых некрозов в почках и печени, нефроза, неспецифического реактивного гепатита. В этих условиях производство АТФ из АДФ становится невозможным, а энергия дыхательной цепи полностью исчезает в виде тепла, это и объясняет жароповышающий и жиросжигающий эффекты динитрофенола.

Acute coronary syndromes typically result from the formation of atherosclerotic lesions in a large epicardial vessel, which restrict blood flow either partially or completely. These lesions can be identified through angiography, an invasive imaging technique that enables visualization of the coronary arteries. However, a small percentage of patients, usually ranging from 5% to 10%, experience symptoms and/or signs of myocardial ischemia, either acute or chronic, without significant obstructive coronary lesions visible on angiography. This condition is particularly prevalent in young women and is characterized by two distinct forms: myocardial infarction with no obstructive coronary arteries (MINOCA) and myocardial ischemia with no obstructive coronary arteries (INOCA). MINOCA can be caused by a variety of heterogeneous mechanisms, including coronary vascular spasm, microvascular disease, spontaneous coronary dissection, and plaque rupture or erosion. Conversely, coronary vasospasm and microvascular dysfunction account for the majority of patients with INOCA. We here present three cases of MINOCA/INOCA that were evaluated using optical coherence tomography, coronary flow reserve, index of microcirculatory resistance, and acetylcholine provocative test. These diagnostic tests allowed us to identify a specific condition and adopt a targeted treatment for each patient.

Cell-to-cell communication must occur through molecular transport in the intercellular fluid space. Nanoparticles, such as exosomes, diffuse or move more slowly in fluids than small molecules. To find a microfluidic technology for real-time exosome experiments on intercellular communication between living cells, we use the microfluidic culture dish\'s quaternary ultra-slow microcirculation flow field to accumulate nanoparticles in a specific area. Taking stem cell-tumor cell interaction as an example, the ultra-slow microcirculatory flow field controls stem cell exosomes to interfere with tumor cells remotely. Under static coculture conditions (without microfluidics), the tumor cells near stem cells (<200 µm) show quick breaking through from its Matrigel drop to meet stem cells, but this \'breaking through\' quickly disappears with increasing distance. In programmed ultra-slow microcirculation, stem cells induce tumor cells 5000 μm far at the site of exosome deposition (according to nanoparticle simulations). After 14 days of programmed coculture, the glomeration and migration of tumor cells were observed in the exosome deposition area. This example shows that the ultra-slow microcirculation of the microfluidic culture dish has good prospects in quantitative experiments to study exosome communication between living cells and drug development of cancer metastasis.

The study aimed to evaluate the relation between microcirculation of the genian region using laser Doppler flowmetry and oral mucositis (OM) and pain in individuals undergoing antineoplastic therapy. A case-control clinical study was conducted with the participants divided into three groups: chemotherapy (CTG), radiation therapy and chemotherapy group (RCTG), and control group (CG). Pain was assessed by visual analog scale; OM was classified by oral mucositis assessment and WHO scales. The blood flow was assessed by laser Doppler flowmetry. Kruskal-Wallis statistical test, Friedman test, and Spearman test were used for statistical analysis of this study. CTG (7 individuals/25.93%) showed the worst manifestations of OM between 2nd and 4th evaluations (OM-WHO: T2, p = 0.006; T3, p = 0.006; T4, p = 0.003; OM-OMAS: T2, p = 0.004; T3, p = 0.000; T4, p = 0.011), and increased blood flow over time, with the exception of the 3rd evaluation (p = 0.138). The RCTG (9 individuals/33.33%) showed the worst manifestations of oral mucositis on the 4th week (OM-WHO and OM-OMAS: p = 0.000) with a decrease in blood flow (p = 0.068). The decreased blood flow is associated with greater severity of oral mucositis and greater pain intensity.

Given that there are controversial findings regarding vessel density in amblyopia, we quantified retinal microcirculation using optical coherence tomography angiography and compared it between hyperopic ametropic amblyopia eyes and age-matched control eyes. This case-control study was conducted from March 2021 to March 2022 at the Affiliated Eye Hospital of Nanchang University, Nanchang, China. Both groups included 72 eyes. Foveal avascular zone area, circularity and perimeter, perfusion density and vessel density of macular superficial retinal capillary plexus, macular thickness, macular volume, peripapillary retinal nerve fiber layer thickness, and ganglion cell-inner plexiform layer thickness were compared between hyperopia ametropic amblyopia eyes and age-matched control eyes. Additionally, best-corrected visual acuity, maximum corneal curvature, minimum corneal curvature, and anterior chamber depth were measured. In the hyperopia ametropic amblyopia eyes and control eyes, vessel density was 7.51 ± 2.13 and 9.91 ± 2.71 mm-1 in the central, 17.20 ± 1.38 and 18.25 ± 1.37 mm-1 in the inner, and 17.90 ± 0.88 and 18.43 ± 0.97 mm-1 in the full regions, respectively. The perfusion densities were 0.17 ± 0.06 and 0.23 ± 0.07 in the central, 0.41 ± 0.05 and 0.44 ± 0.03 in the inner, and 0.44 ± 0.03 and 0.46 ± 0.02 in the full regions, respectively. The central macular thicknesses of hyperopia ametropic amblyopia and control eyes were 240.04 ± 20.11 and 235.08 ± 24.41 µm, respectively. Foveal avascular zone perimeter and circularity (P < .043 and P = .001) significantly differed between the 2 groups. Hyperopia ametropic amblyopia eyes showed lower appreciably in vessel and perfusion densities, which could be one of the major pathophysiological mechanisms of hyperopia ametropic amblyopia and provide a new direction for the diagnosis and treatment of amblyopia.

There are two types of testicular torsion: complete and incomplete. The degree and duration of symptoms of this condition are critical for treatment decision-making, as the consequences for untimely diagnosis and management can be serious. The preoperative assessment of the degree of acute testicular torsion using ultrasonography is particularly important for determining the appropriate intervention. The purpose of this study was to compare the effectiveness of high-frequency versus contrast-enhanced ultrasonography in determining the degree of acute testicular torsion.
Fifteen patients with clinically diagnosed acute testicular torsion underwent both high-frequency and contrast-enhanced ultrasonography. We compared the characteristics of the ultrasonographic images of the testicular parenchyma in both the afflicted and contralateral (healthy) testes to determine the reliability of contrast-enhanced ultrasonography in assessing the degree of acute testicular torsion.
The high-frequency ultrasound and contrast-enhanced ultrasound diagnosis of 4 complete testicular torsion and 11 incomplete testicular torsion were correct before operation. However, 5 patients with incomplete testicular torsion were misdiagnosed as complete testicular torsion because no blood flow was detected by high frequency ultrasound. Finally, low speed blood flow was detected by contrast-enhanced ultrasound and the diagnosis was corrected. The accuracy of diagnosing incomplete testicular torsion was 100% using contrast-enhanced ultrasonography and 66.7% using high-frequency ultrasonography; the difference between the two methods was statistically significant (χ2 = 2.50, P ≤ 0.05).
Contrast-enhanced ultrasonography can diagnose testicular torsion with high accuracy and can detect low-velocity blood flow and show microcirculatory blood perfusion in the testicular parenchyma. This can avoid misdiagnosing incomplete testicular torsion as complete, thus averting unnecessary orchiectomy.

UNASSIGNED: Systemic sclerosis (SSc) is a complex autoimmune disease characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. Treatment with autologous hematopoietic cell transplantation (HCT) for progressive SSc has improved overall and event-free survival rates significantly, but unfortunately disease progression after HCT is seen in a subset of patients. Data on the efficacy and safety of second HCT is scarce.
UNASSIGNED: We present a patient with diffuse cutaneous SSc and associated interstitial lung disease (ILD) who successfully underwent a second HCT for progressive disease five years after a first HCT. We describe changes in skin involvement and pulmonary involvement as well as the changes observed in sequential nailfold microcapillaroscopy (NCM), performed from first presentation up to this moment.
UNASSIGNED: This case adds to the current limited literature on efficacy and safety of a second HCT in SSc refractory cases. Furthermore it outlines the potential of HCT on amelioration of microvasculopathy in SSc.

The patient was a 32-year-old man with no HIV infection and possible syphilis infection at the age of 22 years. At the age of 29 years, he visited an ophthalmologist for diplopia due to right oculomotor nerve palsy. He underwent diplopia strabismus surgery for unexplained oculomotor nerve palsy. At the age of 31 years, he had a left oculomotor nerve palsy and was referred to our department. He was diagnosed with neurosyphilis based on positive serum and cerebrospinal fluid syphilis antibodies. MRI showed aneurysm, asymptomatic cerebral hemorrhage, and contrast enhancement of the left oculomotor nerve, leading to the diagnosis of meningovascular syphilis. The patient\'s symptoms improved with penicillin and corticosteroids. The oculomotor nerve palsy may be due to microcirculatory disorder caused by syphilitic cerebral endarteritis.