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Article ; Online: HB Puerta del Sol [HBA1:c.148A>C], HB Valdecilla [HBA2:c.3G>T], HB Gran Vía [HBA2:c.98T>G], HB Macarena [HBA2:c.358C>T] and HB El Retiro [HBA2:c.364_366dupGTG]: description of five new hemoglobinopathies.

de la Fuente-Gonzalo, Félix / Nieto, Jorge M / Velasco, Diego / Cela, Elena / Pérez, Germán / Fernández-Teijeiro, Ana / Escudero, Antonio / Villegas, Ana / González-Fernández, Fernando A / Ropero, Paloma

Clinical chemistry and laboratory medicine

2016 Volume 54, Issue 4, Page(s) 553–560

Abstract: Background: Structural hemoglobinopathies do not usually have a clinical impact, but they can interfere with the analytical determination of some parameters, such as the glycated hemoglobin in diabetic patients. Thalassemias represent a serious health ... ...

Abstract	Background: Structural hemoglobinopathies do not usually have a clinical impact, but they can interfere with the analytical determination of some parameters, such as the glycated hemoglobin in diabetic patients. Thalassemias represent a serious health problem in areas where their incidence is high. The defects in the post-translational modifications produce hyper-unstable hemoglobin that is not detected by most of electrophoretic or chromatographic methods that are available so far. Methods: We studied seven patients who belong to six unrelated families. The first two families were studied because they had peak abnormal hemoglobin (Hb) during routine analytical assays. The other four families were studied because they had microcytosis and hypochromia with normal HbA2 and HbF without iron deficiency. HbA2 and F quantification and abnormal Hb separation were performed by chromatographic and electrophoretic methods. The molecular characterization was performed using specific sequencing. Results: The Hb Puerta del Sol presents electrophoretic mobility and elution in HPLC that is different from HbA and similar to HbS. The electrophoretic and chromatographic profiles of the four other variants are normal and do not show any anomalies, and their identification was only possible with sequencing. Conclusions: Some variants, such as Hb Valdecilla, Hb Gran Vía, Hb Macarena and Hb El Retiro, have significant clinical impact when they are associated with other forms of α-thalassemia, which could lead to more serious forms of this group of pathologies as for HbH disease. Therefore, it is important to maintain an adequate program for screening these diseases in countries where the prevalence is high to prevent the occurrence of severe forms.
MeSH term(s)	Adult ; Aged, 80 and over ; Child, Preschool ; Chromatography, High Pressure Liquid ; Hematologic Tests ; Hemoglobinopathies/blood ; Hemoglobinopathies/genetics ; Hemoglobins/analysis ; Hemoglobins/chemistry ; Hemoglobins/genetics ; Humans ; Infant ; Male ; Middle Aged
Chemical Substances	Hemoglobins
Language	English
Publishing date	2016-04
Publishing country	Germany
Document type	Journal Article
ZDB-ID	1418007-8
ISSN	1437-4331 ; 1434-6621 ; 1437-8523
ISSN (online)	1437-4331
ISSN	1434-6621 ; 1437-8523
DOI	10.1515/cclm-2015-0649
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Article ; Online: Dilution-Induced Deposition of Concentrated Binary Mixtures of Cationic Polysaccharides and Surfactants.

Fernández-Peña, Laura / Guzmán, Eduardo / Oñate-Martínez, Teresa / Fernández-Pérez, Coral / Ortega, Francisco / Rubio, Ramón G / Luengo, Gustavo S

Polymers

2023 Volume 15, Issue 14

Abstract: This work investigates the effect of dilution on the phase separation process of binary charged polysaccharide-surfactant mixtures formed by two cationic polysaccharides and up to four surfactants of different nature (anionic, zwitterionic, and neutral), ...

Abstract	This work investigates the effect of dilution on the phase separation process of binary charged polysaccharide-surfactant mixtures formed by two cationic polysaccharides and up to four surfactants of different nature (anionic, zwitterionic, and neutral), as well as the potential impact of dilution-induced phase separation on the formation of conditioning deposits on charged surfaces, mimicking the negative charge and wettability of damaged hair fibers. The results obtained showed that the dilution behavior of model washing formulations (concentrated polysaccharide-surfactant mixtures) cannot be described in terms of a classical complex precipitation framework, as phase separation phenomena occur even when the aggregates are far from the equilibrium phase separation composition. Therefore, dilution-enhanced deposition cannot be predicted in terms of the worsening of colloidal stability due to the charge neutralization phenomena, as common phase separation and, hence, enhanced deposition occurs even for highly charged complexes.
Language	English
Publishing date	2023-07-12
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527146-5
ISSN	2073-4360 ; 2073-4360
ISSN (online)	2073-4360
ISSN	2073-4360
DOI	10.3390/polym15143011
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Article ; Online: Efectividad clínica y radiológica del intervalo extendido de dosis con natalizumab en pacientes con esclerosis múltiple recurrente.

García-Estévez, D A / Pérez-Lorenzo, G / Fernández-Pérez, M J / Cid-Rodríguez, C / Ozaita-Arteche, G

Revista de neurologia

2022 Volume 74, Issue 8, Page(s) 265–268

Abstract: Introduction: Natalizumab (NTZ) is a very effective treatment approved for highly active multiple sclerosis. The main risk of treatment with NTZ is the possibility of developing progressive multifocal leukoencephalopathy, which is related to JC virus ... ...

Title translation	Clinical and radiological effectiveness of natalizumab extended dosage interval in patients with relapsing multiple sclerosis.
Abstract	Introduction: Natalizumab (NTZ) is a very effective treatment approved for highly active multiple sclerosis. The main risk of treatment with NTZ is the possibility of developing progressive multifocal leukoencephalopathy, which is related to JC virus positivity and the number of NTZ infusions. This risk decreases with the extended dosage interval (EDI), which involves 9 or fewer infusions/year. However, it is a matter of controversy as to whether EDI remains effective in reducing recurrences and the presence of new lesions in magnetic resonance imaging (MRI). Patients and methods: A prospective observational study was conducted from 1 April 2019 to 30 June 2021, following up patients on NTZ treatment who switched to EDI. Patients should have at least one MRI six months after the start of EDI. The presence of attacks or MRI activity (new lesions in T2) during the EDI was recorded. Results: Twenty-three patients with a mean age of 43.5 ± 9.4 years were included. The median number of NTZ infusions was 68 (minimum, 25; maximum, 127). The median interval between the start of the EDI and the last MRI was 14 months (minimum, 6; maximum, 25), and 23 months from the last medical follow-up visit (minimum, 7; maximum, 28). Two patients (8.7%) presented with attacks and two others (8.7%) showed MRI activity. Conclusions: EDI with NTZ maintains high clinical and activity effectiveness in MRI.
MeSH term(s)	Adult ; Humans ; Immunologic Factors/adverse effects ; Immunologic Factors/therapeutic use ; Leukoencephalopathy, Progressive Multifocal/chemically induced ; Middle Aged ; Multiple Sclerosis/diagnostic imaging ; Multiple Sclerosis/drug therapy ; Multiple Sclerosis, Relapsing-Remitting/diagnostic imaging ; Multiple Sclerosis, Relapsing-Remitting/drug therapy ; Natalizumab/adverse effects ; Natalizumab/therapeutic use ; Recurrence
Chemical Substances	Immunologic Factors ; Natalizumab
Language	Spanish
Publishing date	2022-03-21
Publishing country	Spain
Document type	Journal Article ; Observational Study
ZDB-ID	1468278-3
ISSN	1576-6578 ; 0210-0010
ISSN (online)	1576-6578
ISSN	0210-0010
DOI	10.33588/rn.7408.2021311
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Article ; Online: Dual-energy CT: Technical considerations and clinical applications.

Fernández-Pérez, G C / Fraga Piñeiro, C / Oñate Miranda, M / Díez Blanco, M / Mato Chaín, J / Collazos Martínez, M A

Radiologia

2022 Volume 64, Issue 5, Page(s) 445–455

Abstract: Although dual-energy CT was initially described by Hounsfield in 1973, it remains underused in clinical practice. It is therefore important to emphasize the clinical benefits and limitations of this technique. Iodine mapping makes it possible to quantify ...

Abstract	Although dual-energy CT was initially described by Hounsfield in 1973, it remains underused in clinical practice. It is therefore important to emphasize the clinical benefits and limitations of this technique. Iodine mapping makes it possible to quantify the uptake of iodine, which is very important in characterizing tumors, lung perfusion, pulmonary nodules, and the tumor response to new treatments. Dual-energy CT also makes it possible to obtain virtual single-energy images and virtual images without iodinated contrast or without calcium, as well as to separate materials such as uric acid or fat and to elaborate hepatic iron overload maps. In this article, we review some of the clinical benefits and technical limitations to improve understanding of dual-energy CT and expand its use in clinical practice.
MeSH term(s)	Calcium ; Contrast Media ; Iodine ; Tomography, X-Ray Computed/methods ; Uric Acid
Chemical Substances	Contrast Media ; Uric Acid (268B43MJ25) ; Iodine (9679TC07X4) ; Calcium (SY7Q814VUP)
Language	English
Publishing date	2022-07-21
Publishing country	Spain
Document type	Journal Article ; Review
ISSN	2173-5107
ISSN (online)	2173-5107
DOI	10.1016/j.rxeng.2022.06.003
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Article ; Online: Study of the Dilution-Induced Deposition of Concentrated Mixtures of Polyelectrolytes and Surfactants.

Fernández-Peña, Laura / Guzmán, Eduardo / Fernández-Pérez, Coral / Barba-Nieto, Irene / Ortega, Francisco / Leonforte, Fabien / Rubio, Ramón G / Luengo, Gustavo S

Polymers

2022 Volume 14, Issue 7

Abstract: ... on their behavior in concentrated formulations, less is known on their behavior under practical use conditions, e.g ...

Abstract	Mixtures of polyelectrolytes and surfactants are commonly used in many technological applications where the challenge is to provide well-defined modifications of the surface properties, as is the case of washing formulations in cosmetics. However, if contemporary experimental and theoretical methods can provide insights on their behavior in concentrated formulations, less is known on their behavior under practical use conditions, e.g., under dilution and vectorization of deposits. This makes it difficult to make predictions for specific performance, as, for example, good hair manageability after a shampoo or a comfortable sensorial appreciation after a skin cleanser. This is especially important when considering the formulation of new, more eco-friendly formulations. In this work, a detailed study of the phase separation process induced by dilution is described, as well as the impact on the deposition of conditioning material on negatively charged surfaces. In order to gain a more detailed physical insight, several polyelectrolyte-surfactant pairs, formed by two different polymers and five surfactants that, although non-natural or eco-friendly, can be considered as models of classical formulations, have been studied. The results evidenced that upon dilution the behavior, and hence its deposition onto the surface, cannot be predicted in terms of the behavior of simpler pseudo-binary (mixtures of a polymer and a surfactant) or pseudo-ternary mixtures (two polymers and a surfactant). In many cases, phase separation was observed for concentrations similar to those corresponding to the components in some technological formulations, whereas the latter appeared as monophasic systems. Therefore, it may be assumed that the behavior in multicomponent formulations is the result of a complex interplay of synergistic interactions between the different components that will require revisiting when new, more eco-sustainable ingredients are considered.
Language	English
Publishing date	2022-03-25
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527146-5
ISSN	2073-4360 ; 2073-4360
ISSN (online)	2073-4360
ISSN	2073-4360
DOI	10.3390/polym14071335
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Article ; Online: Orbital supratrochlear fistula secondary to anaesthetic block in cataract surgery.

Díez-Montero, C / Fernández-Pérez, G C / Galindo-Ferreiro, A

Archivos de la Sociedad Espanola de Oftalmologia

2019 Volume 94, Issue 11, Page(s) 561–565

Abstract: A case is described of a woman with history of right peribulbar nerve block prior to cataract surgery that, within a few days, presented with a trochlear intraorbital arteriovenous fistula. This was resolved without sequelae after three months by ... ...

Title translation	Fístula orbitaria supratroclear secundaria a la realización de un bloqueo para anestesia en una cirugía de catarata.
Abstract	A case is described of a woman with history of right peribulbar nerve block prior to cataract surgery that, within a few days, presented with a trochlear intraorbital arteriovenous fistula. This was resolved without sequelae after three months by performing intermittent homolateral carotid massages. This is the first case of supratrochlear intraorbital arteriovenous fistula. There are only 8 cases reported of traumatic intraorbital arteriovenous fistula, and only one was with post-peribulbar anaesthesia.
MeSH term(s)	Aged ; Arteriovenous Fistula/etiology ; Arteriovenous Fistula/therapy ; Cataract Extraction ; Female ; Humans ; Massage/methods ; Nerve Block/adverse effects ; Orbit/blood supply ; Orbital Diseases/etiology ; Orbital Diseases/therapy
Language	Spanish
Publishing date	2019-08-01
Publishing country	Spain
Document type	Case Reports ; Journal Article
ISSN	2173-5794
ISSN (online)	2173-5794
DOI	10.1016/j.oftal.2019.07.005
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Article ; Online: Adrenal pheochromocytoma: Keys to radiologic diagnosis.

Corral de la Calle, M A / Encinas de la Iglesia, J / Fernández-Pérez, G C / Repollés Cobaleda, M / Fraino, A

Radiologia

2022 Volume 64, Issue 4, Page(s) 348–367

Abstract: Pheochromocytomas are adrenal paragangliomas. Potentially malignant, these tumors have a low incidence but clear importance. They can appear in various hereditary syndromes, especially in von Hippel-Lindau syndrome, multiple endocrine neoplasia-2 (MEN2), ...

Abstract	Pheochromocytomas are adrenal paragangliomas. Potentially malignant, these tumors have a low incidence but clear importance. They can appear in various hereditary syndromes, especially in von Hippel-Lindau syndrome, multiple endocrine neoplasia-2 (MEN2), and familial paraganglioma syndromes. In sporadic cases, underlying genetic alterations are often found, and these findings are changing our understanding of the disease. Although these tumors can manifest with a characteristic clinical presentation, in 13.1%-57.6% of cases, it is the radiologist who first suggests the diagnosis, indicating analyses for catecholamines or nuclear medicine examinations. Radiologists should suspect a pheochromocytoma on detection of a well-delimited adrenal mass with rapid, intense enhancement that typically shows cystic and hemorrhagic phenomena, high T2 signal intensity, and the absence of macroscopic or microscopic lipids. The behavior in diffusion-weighted imaging usually does not provide very useful information. Approximately one-third of lesions show late washout similar to that seen with adenomas on CT. Percutaneous puncture should be avoided to avoid the risk of unleashing a severe hypertensive crisis.
MeSH term(s)	Adrenal Gland Neoplasms ; Humans ; Paraganglioma ; Pheochromocytoma ; Syndrome ; von Hippel-Lindau Disease
Language	English
Publishing date	2022-08-10
Publishing country	Spain
Document type	Journal Article
ISSN	2173-5107
ISSN (online)	2173-5107
DOI	10.1016/j.rxeng.2022.07.002
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Article: Relationship between Structural Characteristics of Cellulose Nanocrystals Obtained from Kraft Pulp.

Aguayo, María Graciela / Fernández-Pérez, Arturo / Oviedo, Claudia / Reyes, Guillermo / Reyes-Contreras, Pablo

Nanomaterials (Basel, Switzerland)

2020 Volume 10, Issue 9

Abstract: Kraft pulp cellulose was hydrolyzed using sulfuric acid, under different thermophysical conditions of temperature, time, pulp concentration, and sonication time. The experimental design revealed the effect of these conditions and their interaction on the ...

Abstract	Kraft pulp cellulose was hydrolyzed using sulfuric acid, under different thermophysical conditions of temperature, time, pulp concentration, and sonication time. The experimental design revealed the effect of these conditions and their interaction on the hydrolysis yield obtained. In addition, the top five cellulose nanocrystals (CNCs) yields from this experiment design were analyzed. The results obtained indicated that CNCs possess a morphology that can be described as individualized rod particles, with average diameters less than 50 nm and different size distribution. In the analysis of CNCs features, significant Pearson correlations were established between the crystallinity of the CNC, CNC yield, and interplanar crystallites distance (Δ
Language	English
Publishing date	2020-09-08
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2662255-5
ISSN	2079-4991
ISSN	2079-4991
DOI	10.3390/nano10091775
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Article: Stroke-associated pneumonia according to mCDC criteria: impact on prognosis and antibiotic therapy.

Frontiers in neurology

2024 Volume 15, Page(s) 1358628

Abstract: Objective: The modified Centers for Disease Control and Prevention (mCDC) criteria have been proposed for diagnosing and managing stroke-associated pneumonia (SAP). The objective was to investigate the impact of SAP on stroke outcome depending on ... ...

Abstract	Objective: The modified Centers for Disease Control and Prevention (mCDC) criteria have been proposed for diagnosing and managing stroke-associated pneumonia (SAP). The objective was to investigate the impact of SAP on stroke outcome depending on whether or not it conforms to mCDC criteria. Our secondary objective was to identify the responsible factors for antibiotic initiation in stroke patients. Methods: We conducted a prospective, multicenter, observational study of ischemic stroke patients with moderate to severe stroke (NIHSS≥4) admitted within 24 h. For 7 days, mCDC criteria were assessed daily, and infections and antibiotics were recorded. Pneumonias were divided into those fulfilling mCDC criteria (mCDC-SAP) or not (other pneumonias, OPn). The effect of each type of pneumonia on 3-month outcome was evaluated in separated logistic regression models. Factors associated with antibiotic initiation were explored using a random forest analysis. Results: Of the 342 patients studied, infections were diagnosed in 72 (21.6%), including 39 (11.7%) cases of pneumonia. Of them, 25 (7.5%) fulfilled mCDC criteria. Antibiotics were used in 92% of mCDC-SAP and 64.3% of OPn. In logistic regression analysis, mCDC-SAP, but not OPn, was an independent predictor of poor outcome [OR, 4.939 (1.022-23.868)]. The random forest analysis revealed that fever had the highest importance for antibiotic initiation. Interpretation: The mCDC criteria might be useful for detecting clinically relevant SAP, which is associated with poor outcomes. Isolated signs of infection were more important for antibiotic initiation than compliance with pre-defined criteria. Therefore, adherence to mCDC criteria might result in antibiotic saving without compromising clinical outcome.
Language	English
Publishing date	2024-02-28
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2564214-5
ISSN	1664-2295
ISSN	1664-2295
DOI	10.3389/fneur.2024.1358628
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Article ; Online: Gabapentin Inhibits Multiple Steps in the Amyloid Beta Toxicity Cascade.

González-Sanmiguel, Juliana / Burgos, Carlos F / Bascuñán, Denisse / Fernández-Pérez, Eduardo J / Riffo-Lepe, Nicolás / Boopathi, Subramanian / Fernández-Pérez, Arturo / Bobadilla-Azócar, Catalina / González, Wendy / Figueroa, Maximiliano / Vicente, Benjamín / Aguayo, Luis G

ACS chemical neuroscience

2020 Volume 11, Issue 19, Page(s) 3064–3076

Abstract: ... screening targeting the C terminal region of Aβ, a region involved in the toxic properties of the peptide ...

Abstract	Oligomeric β-amyloid peptide (Aβ) is one of the main neurotoxic agents of Alzheimer's disease (AD). Oligomers associate to neuronal membranes, forming "pore-like" structures that cause intracellular calcium and neurotransmitter dyshomeostasis, leading to synaptic failure and death. Through molecular screening targeting the C terminal region of Aβ, a region involved in the toxic properties of the peptide, we detected an FDA approved compound, gabapentin (GBP), with neuroprotective effects against Aβ toxicity. At micromolar concentrations, GBP antagonized peptide aggregation over time and reduced the Aβ absorbance plateau to 28% of control. In addition, GBP decreased Aβ association to membranes by almost half, and the effects of Aβ on intracellular calcium in hippocampal neurons were antagonized without causing effects on its own. Finally, we found that GBP was able to block the synaptotoxicity induced by Aβ in hippocampal neurons, increasing post-synaptic currents from 1.7 ± 0.9 to 4.2 ± 0.7 fC and mean relative fluorescence intensity values of SV2, a synaptic protein, from 0.7 ± 0.09 to 1.00 ± 0.08. The results show that GBP can interfere with Aβ-induced toxicity by blocking multiple steps, resulting in neuroprotection, which justifies advancing toward additional animal and human studies.
MeSH term(s)	Alzheimer Disease/drug therapy ; Amyloid beta-Peptides/metabolism ; Amyloid beta-Peptides/toxicity ; Animals ; Gabapentin/pharmacology ; Hippocampus/metabolism ; Humans ; Neurons/metabolism ; Peptide Fragments
Chemical Substances	Amyloid beta-Peptides ; Peptide Fragments ; Gabapentin (6CW7F3G59X)
Language	English
Publishing date	2020-09-22
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1948-7193
ISSN (online)	1948-7193
DOI	10.1021/acschemneuro.0c00414
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