[Orthopedic management of localized linear scleroderma in children: a case report]. / Manejo ortopédico de la esclerodermia lineal localizada en niños: reporte de un caso.

INTRODUCTION: Localized scleroderma is the most common form of sclerosis in children; it has an incidence of 2.7 per 100,000 inhabitants, with a predilection for the Caucasian and female races of 2.4 to 4.1 per male. The common denominator is the connective tissue-level condition, causing a variety of clinical presentation ranging from localized sclerotic circumscribed plaques affecting the skin, to deeper conditions that attack muscle and bone tissue, leaving aesthetic consequences and/or disabling deformities in the patient. OBJECTIVE: Case presentation and surgical management. The data presented were obtained from the physical and electronic clinical record, direct interview with patients and family members and monitoring and evaluation of radiographic studies, from January 2012 to November 2017. DISCUSSION: Surgical treatment is indicated when deformities and contractures condition incapacity in the patient. The best results will be obtained once the disease has stopped progressing.

INTRODUCCIÓN: La esclerodermia localizada es la forma de presentación de esclerosis más frecuente en niños; tiene una incidencia de 2.7 por cada 100,000 habitantes, con predilección por la raza caucásica y el sexo femenino con relación de 2.4 a 4.1 por cada varón. El común denominador es la afección a nivel de tejido conectivo, genera una variedad de presentación clínica que va desde placas escleróticas localizadas circunscritas que afectan la piel hasta afecciones más profundas que atacan al tejido muscular y óseo, dejando secuelas estéticas y/o deformidades incapacitantes en el paciente. OBJETIVO: Presentación de caso y su manejo quirúrgico. Los datos expuestos se obtuvieron del expediente clínico físico y electrónico, entrevista directa con paciente y familiares y seguimiento y valoración de estudios radiográficos, desde Enero de 2012 hasta Noviembre de 2017. DISCUSIÓN: El tratamiento quirúrgico está indicado cuando las deformidades y contracturas condicionan incapacidad en el paciente. Los mejores resultados se obtendrán una vez que la enfermedad haya dejado de progresar.

Pb2+ complexes of small-cavity azamacrocyclic ligands: thermodynamic and kinetic studies.

The synthesis, acid-base behavior and Pb2+ coordination chemistry of the new aza-scorpiand like ligand 5-[2-(N-2-fluorenyl)ethylamino]-2,5,8-triaza[9]-2,6-pyridinophane (L1) have been studied by potentiometry, NMR and spectrofluorimetric titrations, and the results are compared with those obtained for the related compounds L2, lacking the fluorenyl group, and L3, the macrocycle lacking the pendant arm. The crystal structures obtained for complexes [PbL1][PbL1Cl](NO3)Cl2·4H2O (1) and [PbL3](ClO4)2 (2) reveal that the metal ion is located over the plane defined by the nitrogen atoms of the macrocyclic core due to its inability to accommodate the large Pb2+ ion in the macrocyclic cavity. For L1, the secondary amino group of the pendant arm is implicated in the coordination of the metal ion, although the stereoactive lone pair of Pb2+ prevents the closed conformation associated with the coordination of metal ions in aza-scorpiand derivatives. The kinetics of the acid-promoted dissociation of the ligand from the Pb2+ complexes with the three ligands have been studied using stopped-flow with simultaneous absorbance and fluorescence detection. The results indicate that in spite of their similarity, the dissociation of the metal ion occurs with very different rates in the three complexes. During the course of the kinetic studies evidence was obtained for the occurrence of a photochemical process that leads to ligand degradation with the unexpected elimination of one CH2CH2 fragment from the macrocyclic core.

Bicyclo[2.2.2]octane-1,4-dicarboxylic acid: towards transparent metal-organic frameworks.

The preparation of transparent porous materials can offer a different access towards the study of molecules under solid confined space. Metal-organic frameworks represent a unique opportunity due to their tunable pore size, however aromatic linkers present strong absorption and reduce the transparency. Herein, we report the first example of a MOF with bicyclic organic dicarboxylic linkers and its use as a solid solvent.

[Minimally invasive reconstruction of the posterolateral corner with simultaneous replacement of the anterior cruciate ligament for complex knee ligament injuries]. / Reconstrucción mínimamente invasiva de la esquina posterolateral con sustitución simultánea del ligamento cruzado anterior para lesiones ligamentarias complejas de rodilla.

OBJECTIVE: To evaluate and describe the results of a simultaneous reconstruction with minimally invasive technique of the posterolateral complex (PLC) and the anterior cruciate ligament (ACL). MATERIAL AND METHODS: ACL and PLC reconstruction was performed in seven patients using the technique described, in the period from March to November 2012. All patients were evaluated at six months after the procedure using IKDC and IKSS subjective tests. Their return to work activities and their level of satisfaction were assessed. RESULTS: Six male and one female patients ranging in age between 26 and 46 years were evaluated. The injuries were mostly caused by sports related accidents. All patients were economically active and required an average period of three months of disability. The assessment and outcomes at six months, according to the IKDC scale, were: one patient with IKDC A, four with IKDC B, one patient with C, and one with D. In the subjective scale IKSS, 80% averaged a knee stability of over 90 points, a patient had a 100-point scale and another, of 70 points.

INTRODUCCIÓN: Es importante conocer la efectividad del uso de técnicas combinadas para el tratamiento de las lesiones ligamentarias complejas de la rodilla, su evolución y las estrategias utilizadas para su desarrollo. OBJETIVO: Evaluar y describir los resultados de reconstruir de manera simultánea con una técnica mínimamente invasiva el complejo posterolateral (CPL) y el ligamento cruzado anterior (LCA). MATERIAL Y MÉTODOS: Se realizó la reconstrucción del LCA y el CPL en siete pacientes, mediante la técnica descrita, en el período de Marzo a Noviembre de 2012. Todos los pacientes se evaluaron a los seis meses del procedimiento a través del uso de pruebas subjetivas IKDC, IKSS. Se valoró su retorno a actividades laborales y su índice de satisfacción. RESULTADOS: Seis pacientes fueron del sexo masculino y solo uno del femenino, con rangos de edad entre los 26 y 46 años. La mayoría de las lesiones fueron causadas por accidentes asociados al deporte. Todos los participantes eran personas en la etapa productiva de la vida que requirieron un período promedio de tres meses de incapacidad. A los seis meses, conforme a la escala de IKDC, obtuvimos un paciente con IKDC de A, cuatro con un IKDC de B, uno de C y uno de D. En la escala subjetiva de IKSS, 80% promedió estabilidad de la rodilla por arriba de los 90 puntos, un paciente tuvo escala de 100 puntos y otro de 70 puntos.

Rabdomiosarcoma alveolar orbitario enmascarado por sinusitis etmoidal en un adulto de 25 años / Orbital alveolar rhabdomyosarcoma masked by ethmoid sinusitis in a 25-year-old

CASO CLÍNICO: Mujer de 25 años con sinusitis subaguda derecha acudió por molestias en ojo derecho. Ante clínica y tomografía urgente sugestiva de absceso subperióstico orbitario etmoidal, se realizó un drenaje urgente, hallándose una tumoración sólida con biopsia positiva para rabdomiosarcoma alveolar. Se inició tratamiento con quimioterapia y radioterapia con remisión completa y resolución del cuadro clínico. DISCUSIÓN: El rabdomiosarcoma orbitario alveolar en adultos es infrecuente. Presenta un alto riesgo de diseminación y puede debutar simulando una sinusitis, como en este caso, por lo que un diagnóstico y tratamiento precoces son de especial importancia en estos pacientes

CASE REPORT: A 25-year-old woman with right subacute sinusitis, complained about discomfort in her right eye. Clinical manifestations and computed tomography were suggestive of sub-periosteal orbital ethmoid wall abscess, for which the patient underwent urgent drainage. A solid tumor was found, with a positive biopsy for alveolar rhabdomyosarcoma. Complete remission and resolution of orbital symptoms were achieved with chemotherapy and radiation therapy. DISCUSSION: Alveolar orbital rhabdomyosarcoma in adults is uncommon. Rhabdomyosarcoma has a high risk of spreading. It can simulate a sinusitis, as in our patient, early diagnosis and early treatment being especially important in these patients

Xantogranuloma necrobiótico. Diagnóstico diferencial, tratamiento e implicaciones sistémicas. A propósito de un caso / Necrobiotic xanthogranuloma. Differential diagnosis, treatment and systemic involvement. Case report

CASO CLÍNICO: Varón de 48 años remitido por edema palpebral con nódulos ulcerados amarillentos en ambos párpados superiores. Las técnicas de imagen demostraron infiltración en ambas órbitas anteriores que fueron biopsiadas con el resultado de xantogranuloma necrobiótico. En el estudio sistémico se halló una gammapatía monoclonal IgG no conocida. Se inició tratamiento con corticoides y ciclosporina orales sin éxito, por lo que se pautaron inyecciones intralesionales de triamcinolona que controlaron la enfermedad orbitaria. Discusión: El xantogranuloma necrobiótico es una rara entidad que suele afectar a párpados y órbita anterior. Su diagnóstico es importante porque se asocia a procesos linfoproliferativos malignos. Los corticoides intralesionales fueron efectivos en su manejo

CASE REPORT: A 48-year-old male was referred to our hospital for further evaluation of eyelid edema with bilateral yellowish ulcerated nodules. Suspecting a xanthogranulomatosis, imaging tests and biopsy were performed with diagnosis of necrobiotic xanthogranuloma. IgG monoclonal gammapathy was diagnosed in a systemic study. Systemic corticosteroids and cyclosporine were initiated unsuccessfully; therefore intra-lesional injections of triamcinolone were started, which controlled the orbital disease. Discussion: Necrobiotic xanthogranuloma is a rare condition that usually affects the eyelids and anterior orbit. Its diagnosis is important as it is associated with malignant lymphoproliferative processes. Intra-lesional corticosteroids were effective in our patient

In vitro leishmanicidal activity of pyrazole-containing polyamine macrocycles which inhibit the Fe-SOD enzyme of Leishmania infantum and Leishmania braziliensis species.

The in vitro leishmanicidal activity and cytotoxicity of pyrazole-containing macrocyclic polyamines 1-4 was assayed on Leishmania infantum and Leishmania braziliensis species. Compounds 1-4 were more active and less toxic than glucantime and both infection rates and ultrastructural alterations confirmed that 1 and 2 were highly leishmanicidal and induced extensive parasite cell damage. Modifications in the excretion products of parasites treated with 1-3 were also consistent with substantial cytoplasm alterations. Compound 2 was highlighted as a potent inhibitor of Fe-SOD in both species, whereas its effect on human CuZn-SOD was poor. Molecular modelling suggested that 2 could deactivate Fe-SOD due to a sterically favoured enhanced ability to interact with the H-bonding net that supports the enzyme`s antioxidant features.

[Necrobiotic xanthogranuloma. Differential diagnosis, treatment and systemic involvement. Case report]. / Xantogranuloma necrobiótico. Diagnóstico diferencial, tratamiento e implicaciones sistémicas. A propósito de un caso.

CASE REPORT: A 48-year-old male was referred to our hospital for further evaluation of eyelid edema with bilateral yellowish ulcerated nodules. Suspecting a xanthogranulomatosis, imaging tests and biopsy were performed with diagnosis of necrobiotic xanthogranuloma. IgG monoclonal gammapathy was diagnosed in a systemic study. Systemic corticosteroids and cyclosporine were initiated unsuccessfully; therefore intra-lesional injections of triamcinolone were started, which controlled the orbital disease. DISCUSSION: Necrobiotic xanthogranuloma is a rare condition that usually affects the eyelids and anterior orbit. Its diagnosis is important as it is associated with malignant lymphoproliferative processes. Intra-lesional corticosteroids were effective in our patient.

[Orbital alveolar rhabdomyosarcoma masked by ethmoid sinusitis in a 25-year-old]. / Rabdomiosarcoma alveolar orbitario enmascarado por sinusitis etmoidal en un adulto de 25 años.

CASE REPORT: A 25-year-old woman with right subacute sinusitis, complained about discomfort in her right eye. Clinical manifestations and computed tomography were suggestive of sub-periosteal orbital ethmoid wall abscess, for which the patient underwent urgent drainage. A solid tumor was found, with a positive biopsy for alveolar rhabdomyosarcoma. Complete remission and resolution of orbital symptoms were achieved with chemotherapy and radiation therapy. DISCUSSION: Alveolar orbital rhabdomyosarcoma in adults is uncommon. Rhabdomyosarcoma has a high risk of spreading. It can simulate a sinusitis, as in our patient, early diagnosis and early treatment being especially important in these patients.

Persistence of the efficacy of copper oxide wire particles against Haemonchus contortus in sheep.

The aim was to determine the persistent efficacy of copper oxide wire particles (COWP) against Haemonchus contortus in sheep, using the harmonization guidelines protocol. Thirty-six male lambs (2 months old) reared free of gastrointestinal nematodes were used (average body weight of 10.8±3.8kg). Before and for the duration of the study, lambs were kept in raised cages with slatted floors and were offered ad libitum a complete mixed diet. Animals were divided into six groups (n=6): one non-treated control group (G0) and five groups treated with one COWP capsule (1.7g of copper oxide; Copinox(®)). Animals in each group were treated on pre-defined dates before the artificial infection was applied: days -35 (G1), -28 (G2), -21 (G3), -14 (G4) and -7 (G5). On day 0 animals were infected with 3700 H. contortus infective larvae per animal. Animals were humanely slaughtered between days 22 and 23 post-infection. The abomasums were individually washed to obtain the contents. These organs were subjected to separate artificial digestions. Adult parasites were counted from the abomasum contents and the larvae from the digested material. Worm burden geometric means were calculated for each group. A significant worm burden reduction in either of the treated groups (G1, G2, G3, G4, and G5) compared to the control (G0) was considered as persistence of the anthelmintic effect. Copper levels were determined from individual liver samples of each animal. The geometric mean worm burden of the control group (G0) was 1959. Compared to the control, worm burdens geometric means were significantly reduced in groups G1 (1108), G4 (528) and G5 (1063) (P<0.03). Efficacies in G1, G4 and G5 were 43.4%, 73.0% and 45.7% respectively. No significant reduction was found for G2 (1342) and G3 (1430). A larger quantity of Cu was found in the livers of treated animals compared to the control group (P<0.05) except for G3 (P=0.06). A negative association between Cu liver content and worm burdens was found (r=-0.42, P<0.05). Live weight gain was similar in all groups and no clinical or post-mortem manifestations of Cu toxicity were recorded in treated animals.

Confocal microscopy study of donor-recipient interface after Descemet's stripping with endothelial keratoplasty.

AIM: To evaluate the in vivo confocal microscopic corneal features seen in patients who underwent Descemet's stripping with endothelial keratoplasty (DSEK) and to correlate these findings with the postoperative best spectacle-corrected visual acuity (BSCVA) obtained at the time of confocal microscopy. METHODS: Observational case series. A review of charts and confocal microscopy images and videos (Confoscan 4; Nidek Technologies America, Greensboro, North Carolina, USA) of 25 consecutive eyes from 21 patients who underwent DSEK for Fuchs' dystrophy or pseudophakic bullous keratopathy and had a follow-up of at least 6 months post-DSEK. Time from DSEK to confocal microscopy ranged from 6 to 22 months. Subepithelial haze, donor-recipient interface haze, interface birefringent particles and presence of stromal folds were evaluated by a blinded observer and correlated to the postoperative BSCVA. RESULTS: 25 eyes (21 patients) with a mean age of 71.3 (11.8) years were included. A significant negative correlation was found between postoperative BSCVA and subepithelial corneal haze (Spearman correlation coefficient) (r(s)=-0.651; p=0.0004). Donor-recipient interface haze (r(s)=-0.0908; p=0.6658), interface particles (r(s)=-0.2403; p=0.2472) or the presence of stromal folds (r(s)=0.111; p=0.6055) did not correlate with BSCVA. CONCLUSION: Subepithelial haze, donor-recipient stromal interface haze, birefringent particles and stromal folds were present at different degrees in all patients who underwent DSEK. However, subepithelial haze was the only factor that correlated to postoperative BSCVA.

Caso atípico de edema agudo de pulmón en el postparto no inmediato / Atypical case of cute pulmonary edema in non-immediate postpartum

Describimos el caso de una mujer joven sin antecedentes previos de interés, que tras su 5º parto vía vaginal y que transcurrió sin incidencias tanto el embarazo como el parto, ingresa 1 semana después por un cuadro de edema agudo de pulmón con insuficiencia respiratoria hipoxémica severa secundaria y datos de hipertensión arterial pulmonar, muy bien toleradoclínicamente y autolimitado. Discutimos los posibles diagnósticos diferenciales del edema agudo pulmonar postparto y en relación con su fisiopatología las posibles causas que justificaran nuestro caso, sin etiología evidente tras los estudios realizados. Anuestro conocimiento tras revisión de la literatura es un caso excepcional la situación de edema agudo de pulmón con hipertensión arterial pulmonar en el postparto no inmediato, y autolimitado

We describe the case of a young woman with no previous background of interest who one week after after her 5th vaginal delivery and having had a pregnancy and delivery with no incidences was admitted to hospital due to an acute pulmonary edema picture with severe secondary hypoxemic respiratory failure and data of pulmonary arterial hypertension, that was very well tolerated clinically and was self-limited. We discuss the possible differential diagnoses of acute postpartum pulmonary edema and, regarding its pathophysiology, the possible causes that would justify our case,with no clear etiology after the studies conducted. To our knowledge, after the review of the literature, the situation of acute pulmonary edema with pulmonary arterial hypertension in the non-immediate postpartum that is self-limited is a rare case26183

Oxaaza cyclophanes in the recognition of nucleotides. The role of oxygen and electron-rich aromatic rings.

Dioxapolyaza cyclophanes derived from resorcinol and different polyamine chains have been studied in aqueous solution as abiotic receptors for nucleotides. The presence of the additional ethyleneoxy subunits is reflected in a higher basicity and in a significant increase in the log K values for the interaction with nucleotides relative to that of related polyazacyclophanes.

[Atypical ophthalmic findings in Susac syndrome]. / Síndrome de Susac de presentación ocular atípica.

CASE REPORT: A 25-year-old woman with encephalopathy and sensorineural hearing loss was referred to us in order to confirm Susac syndrome, due to bilateral visual loss. Funduscopic examination revealed peripheral retinal microangiopathy. DISCUSSION: Susac syndrome is typically characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. In our case, ophthalmic findings were atypical. Interrupted vessels and microaneurisms accumulation in the peripheral retina of both eyes were observed.

Síndrome de Susac de presentación ocular atípica / Atypical Ophtalmic Findings in Susac Syndrome

Caso clínico: Mujer de 25 años de edad afecta de encefalopatía y pérdida auditiva neurosensorial, remitida a nuestro servicio para confirmar un síndrome de Susac por presentar una disminución de agudeza visual bilateral. En la exploración funduscópica se apreció una microangiopatía retiniana periférica. Discusión: El síndrome de Susac se caracteriza típicamente por la tríada encefalopatía, perdida auditiva neurosensorial, y oclusiones de ramas arteriales retinianas. En nuestro caso las alteraciones oculares eran atípicas, apreciándose amputación de vasos terminales con cúmulo de microaneurismas en retina periférica de ambos ojos (AU)

Case report: A 25-year-old woman with encephalopathy and sensorineural hearing loss was referred to us in order to confirm Susac syndrome, due to bilateral visual loss. Funduscopic examination revealed peripheral retinal microangiopathy. Discussion: Susac syndrome is typically characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. In our case, ophthalmic findings were atypical. Interrupted vessels and microaneurisms accumulation in the peripheral retina of both eyes were observed (AU)

Urrets-Zavalia syndrome as a complication of argon laser peripheral iridoplasty.

OBJECTIVE: To report on 8 patients who developed prolonged mydriasis after argon laser peripheral iridoplasty (ALPI). DESIGN: Retrospective case series. METHODS: A review of the charts of 8 patients with persistent occludable angles after laser iridotomy who developed mydriasis after undergoing ALPI. RESULTS: 12 eyes of 8 patients (mean age 50.2, standard deviation 6.9, range 40-61 years) developed persistent dilatation. 7 of 8 patients had plateau iris syndrome. Only one patient had a decrease in visual acuity. Intraocular pressure increased only in 1 eye and remained stable or decreased in the others. Of the 8 patients, 7 had blurred vision, 2 had photophobia, 4 had glare and 1 had discomfort. The pupillary response to pilocarpine instillation was minimal or absent. Mydriasis eventually resolved in all eyes without treatment. CONCLUSION: ALPI can be complicated by mydriasis unresponsive to pilocarpine. Mydriasis and accompanying symptoms resolved spontaneously within 1 year in most patients.

Aplicaciones clínicas de la determinación plasmática del péptido natriurético auricular / Clinical applications of auricular natriuretic peptide plasma level quantification

El péptido natriurético auricular tipo B (BNP) es una neurohormona sintetizada en los ventrículos. En el momento actual, la medida del BNP se puede realizar mediante una rápida prueba que está disponible tanto en el ámbito ambulatorio como en el hospitalario. Se ha probado su utilidad en el Servicio de Urgencias para el diagnóstico de la insuficiencia cardíaca congestiva en pacientes con causa no clara de disnea. Se ha estudiado la utilidad de la determinación del nivel de BNP como criterio de ingreso hospitalario, así como en la toma de decisiones respecto a la adecuación del tratamiento durante la hospitalización y el momento del alta. En pacientes ambulatorios, usar el BNP como guía para intensificar la terapéutica farmacológica muestra un prometedor futuro en la disminución de la tasa de eventos adversos asociados con este diagnóstico. La medida del BNP parece también proporcionar información pronóstica en pacientes con insuficiencia cardíaca, así como ser útil en la detección de la disfunción ventricular izquierda asintomática. Además, se intuye su utilidad en otros campos como en el síndrome coronario agudo y el corazón trasplantado, sin olvidar el uso reciente del BNP recombinante humano, nesiritide, en el tratamiento de la insuficiencia cardíaca descompensada (AU)

Keratolimbal allograft in corneal reconstruction.

The replenishment of corneal epithelial SC is a crucial step for reconstructing the ocular surface in patients suffering from devastating ocular surface diseases manifesting with total LSCD. KLAL is one of such procedures and has a long track record and a long follow-up for patients with bilateral total LSCD. This review summarizes the literature experiences and outline new strategies that are important to enhance the success of this procedure. Further research is needed to fully understand the biological processes involved in allogeneic tissue transplantation for preserving epithelial SC adhesion, migration, and survival.

Rat enterocyte cultures on human amniotic membranes.

BACKGROUND: Rat enterocytes were cultured on human amniotic membranes. METHODS: Intestine of neonatal DA rats was digested using collagenase and dispase according to the technique developed by Evans. The harvested enterocytes were cultured on human amniotic membranes using standard cell culture techniques. RESULTS: After the second day of culture, some intestinal epithelial units started to gradually detach from the membrane, dispersing as single cells and disappearing within a few days. On the contrary, other units showed signs of cell proliferation. The cultured cells underwent morphologic changes, survived, and remained attached to the amniotic membrane for 3 weeks. Paraffin sections of the membrane showed cultured cytokeratin-positive cells attached to the membrane as a monolayer. CONCLUSIONS: Human amniotic cell membranes help to maintain rat enterocytes in culture for a long time period (3 weeks), possibly via secretion of trophic factors. This technique may provide a valuable tool to study the development and the properties of these epithelial cells in a culture environment.