Clinical and economic burden of acute exacerbations of idiopathic pulmonary fibrosis: a prospective observational study in Spain (OASIS study).

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a fatal progressive lung disease entailing significant impairment in health-related quality of life (HRQoL) and high socioeconomic burden. The course of IPF includes episodes of acute exacerbations (AE-IPF) leading to poor outcomes. This study aimed to compare management, costs and HRQoL of patients with AE-IPF to patients without AE-IPF during one year in Spain. MATERIALS AND METHODS: In a 12-month, prospective, observational, multicenter study of IPF patients, healthcare resource use was recorded and costs related to AE-IPF were estimated and compared between patients with and without AE-IPF. HRQoL was measured with the St. George's Respiratory Questionnaire (SGRQ), EuroQoL 5 dimensions 5 levels questionnaire (EQ-5D-5L), EQ-5D visual analogue scale (EQ-VAS) and the Barthel Index. RESULTS: 204 IPF patients were included: 22 (10.8%) experienced ≥ 1 acute exacerbation, and 182 (89.2%) did not. Patients with exacerbations required more primary care visits, nursing home visits, emergency visits, hospital admissions, pharmacological treatments and transport use (p < 0.05 for all comparisons). Likewise, patients with exacerbations showed higher annual direct health AE-IPF-related costs. In particular, specialized visits, emergency visits, days of hospitalization, tests, palliative care, transport in ambulance and economic aid (p < 0.05 for all comparisons). Exploratory results showed that patients with AE-IPF reported a non-significant but substantial decline of HRQoL compared with patients without AE-IPF, although causality can be inferred. CONCLUSION: We observed significantly higher resource use and cost consumption and lower HRQoL among patients suffering exacerbations during the study. Thus, preventing or avoiding AE-IPF is key in IPF management.

Post-traumatic retinal siderosis, a case report.

A thirty-year-old patient attended a few hours after an ocular trauma while hammering, receiving trauma with a metal splinter at the left eye. Due to an unfavorable clinical picture, surgical management was decided, which was initially rejected by the patient. He returned six months later with a profound decrease in left eye visual acuity, reaching counting finger at one meter despite optical correction. The anterior segment shows a lower scarring leukoma, associated with Tyndall (++), retrokeratic pigment, lower posterior synechiae and a total cataract. Cataract surgery with intraocular lens implantation and a posterior vitrectomy with intraocular foreign body extraction were indicated. At postoperative control it was shown that post-traumatic ocular siderosis did not significantly affect his central vision, which remains until now.

Comparison of water- vs. land-based exercise for improving functional capacity and quality of life in patients living with and beyond breast cancer (the AQUA-FiT study): a randomized controlled trial.

BACKGROUND: Patients living with and beyond breast cancer frequently exhibit several side effects that can impact quality of life and physical functioning way beyond diagnosis and cancer therapies. Traditional on-land exercise has shown to be effective in reducing several symptoms of BC but little is known about the role of water-based exercise in improving physical and psychological well-being. OBJECTIVES: To compare land- vs. water-based exercise training for BC survivors to improve Health-Related Quality of Life (HRQoL), cancer-related fatigue (CRF), physical functioning, body composition and physical activity in patients with BC. METHODS: A randomised, parallel group (1:1) controlled trial was conducted between 2020 and 2022. Patients were randomly allocated to complete a similar exercise training twice weekly during 12 weeks either on land (LG) using traditional gym equipment or in a swimming pool (WG) using body-weight exercises and water-suitable accessories. Both groups were supervised and monitored by an experienced physiotherapist. Main outcome was HRQoL (EORTC QLQ C30 and B23 module) and CRF measured with the Piper Scale. Secondary variables included functional capacity with the 6 Minutes Walking Test (6MWT), upper and lower body strength (handgrip strength and 30″ Sit-to-Stand (STS) test), body composition and objectively measured physical activity. RESULTS: 28 patients were assessed and randomised during the study period. One patient did not receive the allocated intervention due to skin issues and one patient was dropped out during the intervention. A significant effect of time was found for both symptom severity (F(2,52) = 6.46, p = 0.003) and overall functioning (F1.67,43.45 = 5.215, p =0 .013) but no interaction was found between group and time. No effects were reported for CRF. Similar findings were reported for functional capacity (time effect F1.231,32.019 = 16.818, p < 0.001) and lower body strength (time effect F2,52 = 15.120, p < 0.001) as well as fat mass (time effect F2,52 = 4.38, p = 0.017). Notably, a significant time per group interaction was reported for physical activity (F2,52 = 6.349, p =0.003) with patients in the WG significantly improving PA levels over time while patients in the LG exhibited a marked decreased. CONCLUSIONS: Exercise training either in water or on land can decrease symptom severity and improve functionality and body composition. Water-based training seems more effecting than land-based exercise to improve physical activity patterns over time.

Clinical and Surgical Outcomes of Endoscopic Endonasal Approach for Giant Pituitary Adenomas: Analysis of Predictive Factors.

BACKGROUND: Giant pituitary adenomas (GPAs) are defined as tumors with ≥40 mm in any maximum diameter, and these tend to invade multiple intracranial compartments. Hence, treatment remains a surgical challenge. OBJECTIVE: To describe the clinical and surgical outcomes of the endoscopic endonasal approach (EEA) for GPA in a pituitary referral center in Latin America and to analyze associated predictive factors. METHODS: 37 patients with histologically-confirmed GPA treated solely through the EEA between a 2-year period were included. Preoperative and postoperative clinical and neuroimaging findings; surgical morbidity and mortality; and binary logistic regression analysis to assess predictive factors were analyzed. RESULTS: Preoperative visual impairment prevalence was 97.3%. Mean tumor volume was 32 cc and gross total resection rate was 40.5%. Favorable visual acuity and visual fields outcome rate was 75% and 82.9%, respectively. In the multivariate analysis, bilateral cavernous sinus invasion (P = 0.018) and postoperative cerebrospinal fluid (CSF) leak (P = 0.036) were associated with an unfavorable visual acuity outcome, while radiation therapy (P = 0.035) was for visual fields. Similarly, intraoperative CSF leak was a predictive factor for postoperative CSF leak (10.8%) (P = 0.042) and vascular injury (13.5%) (P = 0.048). CONCLUSIONS: In this first Mexican clinical series, we demonstrated that the EEA is a safe and effective technique for GPA, although early diagnosis and prompt intervention may promote further visual function preservation without significant endocrine morbidity.

Orbital ophthalmomiasis due to Dermatobia hominis in an immunosuppressed patient from the ecuadorian andean area.

An 83-year-old Ecuadorian male, indigent with a history of malnutrition and chronic alcoholism, presented with a clinical feature characterized by progressive growth of larvae at the left eye level (LE), associated with areas of ocular tissue putrefaction. Upon admission, his vision in the right eye (RE) was of hand movement and no light perception in LE, the anterior segment of the RE showed a brunescent cataract, with no other alteration, while the LE presented edema, bipalpebral erythema and an abundant number of mobile larvae distributed throughout the orbit, associated with areas of necrosis, putrefaction and distortion of the anatomy of the ocular tissues. Orbital tomography showed a significant compromise of the orbit, indicating surgical management by left orbital exenteration with taxonomic identification of the larvae as Dermatobia hominis associated with it, antibiotic treatment was implemented and a frontal fasciocutaneous flap was performed by the plastic surgery department.

State of refractive surgery with Visian ICL posterior chamber phakic lens in Spain.

OBJECTIVES: To analyse the state of refractive surgery with Visian ICL phakic lens implantation in Spain, indicating the mean ranges of implanted powers, visual and refractive outcomes obtained and safety and efficacy indices. METHOD: This is a population-based, cross-sectional, descriptive, analytical, retrospective, multicentre study in which each co-author collected data from at least the last 10 patients (up to a maximum of 30) with Visian ICL phakic lens implantation. One eye from each subject was randomly selected for study inclusion, and the variables analysed were: age, gender, visual acuities with and without correction pre and post-surgery, preoperative and residualrefraction, keratometry, endothelial cellular density, anterior chamber depth from endothelium, central corneal pachymetry, intraocular pressure, white-white, implanted lens (power and size), expected and achieved spherical equivalent (SEQ) post-surgery at one month after surgery. RESULTS: A sample of 140 eyes was obtained from 140 patients with a mean age of 31.35 ± 7.28 years. Mean preoperative SEQ was -6.33 ± 3.69D, mean CDVA was 0.96 ± 0.16, ACD was 3.30 ± 0.29 mm, WTW 12.02 ± 0.40 mm, CCT 535.03 ± 37.68 µm, ECD 2684.37 ± 313.74 cels/mm2 and IOP 14.84 ± 2.59 mmHg. 66.4% of the implanted lenses were spherical ICLs and 33.6% toric, with a mean power of -7.81 ± 4.09D and a mean cylinder of 2.27 ± 1.23D. 5.7% of the implanted lenses were hypermetropic. The mean SEQ target selected was +0.04 ± 0.27D. 48.92% of the implanted lenses were of size 13.2 mm, with the remaining 36.69, 10.79 and 3.60% being of sizes 12.6, 12.1 and 13.7 mm, respectively. At one month after surgery a residual SEQ of -0.01 ± 0.31D was obtained. The efficacy and safety indices were 1.06 ± 0.18 and 1.10 ± 0.19, respectively. The mean central vault was 508.80 ± 201.04 µm. The accuracy between the calculated SEQ and the obtained SEQ was 99.3% for spherical lenses and 88.2% for Cyl correction. The mean ECD at 1 month after surgery was 2805.53±273.30céls/mm2, which was a difference from preoperative of 0.11% (p = 0.922), similarly, the mean IOP was 13.98 ± 2.57 mmHg, which was a difference of -0.92 ± 2.80 mmHg. CONCLUSIONS: ICL phakic lens surgery is an effective, safe and predictable procedure. There were no significant changes in endothelial cell count or intraocular pressure measurement one month after surgery.

Short-term decreased post transplant lymphoproliferative disorder risk after kidney transplantation using two novel regimens.

BACKGROUND: Belatacept is employed alongside calcineurin inhibitor (CNI) therapy to prevent graft rejection in kidney transplant patients who are Epstein-Barr virus (EBV) seropositive. Preliminary data suggested that rates of post-transplant lymphoproliferative disorder (PTLD) were higher in individuals treated with belatacept compared to CNI therapy alone. METHODS: The records of 354 adults who underwent kidney only transplantation from January 2015 through September 2021 at one medical center were evaluated. Patients underwent treatment with either low-doses of mycophenolate, tacrolimus and sirolimus (B0, n = 235) or low-doses of mycophenolate, tacrolimus and belatacept (B1, n = 119). All recipients underwent induction with antithymocyte globulin and a rapid glucocorticosteroid taper. Relevant donor and recipient information were analyzed and endpoints of PTLD were assessed. RESULTS: There were no cases of PTLD in either cohort within the study period. Recipients in the belatacept cohort experienced lower estimated glomerular filtration rates at 12 months (B0: 67.48 vs. B1: 59.10, p = 0.0014). Graft failure at 12 (B0: 1.28% vs. B1: 0.84%, p = 1.0) and 24 months (B0:2.55% vs. B1: 0.84%, p = 0.431) were similar. There was no difference in rejection rates at 12 (B0: 1.27% vs. B1: 2.52%, p = 0.408) or 24 months (B0: 2.12% vs. B1: 2.52%, p = 1.000). Both groups had similar rates of malignancy, mortality and CMV/BK viremia. CONCLUSION: Non-belatacept (MMF, tacrolimus and sirolimus) and belatacept-based (MMF, tacrolimus and belatacept) regimens do not appear to pose any increased risk of early onset PTLD. Both cohorts benefited from low rates of rejection, malignancy, mortality and graft failure. Recipients will continue to be monitored as PTLD can manifest as a long-term complication.

ACR Appropriateness Criteria® Low Back Pain: 2021 Update.

In the United States, acute low back pain, with or without radiculopathy, is the leading cause of years lived with disability and the third ranking cause of disability-adjusted life-years. Uncomplicated acute low back pain and/or radiculopathy is a benign, self-limited condition that does not warrant any imaging studies. Imaging is considered in those patients who have had up to 6 weeks of medical management and physical therapy that resulted in little or no improvement in their back pain. It is also considered for those patients presenting with red flags, raising suspicion for a serious underlying condition, such as cauda equina syndrome, malignancy, fracture, or infection. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Plexopathy: 2021 Update.

Plexopathy may be caused by diverse pathologies, including trauma, nerve entrapment, neoplasm, inflammation, infection, autoimmune disease, hereditary disease, and idiopathic etiologies. For patients presenting with brachial or lumbosacral plexopathy, dedicated plexus MRI is the most appropriate initial imaging modality for all clinical scenarios and can identify processes both intrinsic and extrinsic to the nerves. Other imaging tests may be appropriate for initial imaging depending on the clinical scenario. This document addresses initial imaging strategies for brachial and lumbosacral plexopathy in the following clinical situations: nontraumatic plexopathy with no known malignancy, traumatic plexopathy (not perinatal), and plexopathy occurring in the context of a known malignancy or posttreatment syndrome. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Suspected Spine Infection.

Spine infection is both a clinical and diagnostic imaging challenge due to its relatively indolent and nonspecific clinical presentation. The diagnosis of spine infection is based upon a combination of clinical suspicion, imaging evaluation and, when possible, microbiologic confirmation performed from blood cultures or image-guided percutaneous or open spine biopsy. With respect to the imaging evaluation of suspected spine infection, MRI without and with contrast of the affected spine segment is the initial diagnostic test of choice. As noncontrast MRI of the spine is often used in the evaluation of back or neck pain not responding to conservative medical management, it may show findings that are suggestive of infection, hence this procedure may also be considered in the evaluation of suspected spine infection. Nuclear medicine studies, including skeletal scintigraphy, gallium scan, and FDG-PET/CT, may be helpful in equivocal or select cases. Similarly, radiography and CT may be appropriate for assessing overall spinal stability, spine alignment, osseous integrity and, when present, the status of spine instrumentation or spine implants. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Neisseria gonorrhoeae: un patógeno díscolo: conceptos microbiológicos, resistencia a antimicrobianos y su vigilancia epidemiológica en Chile / Neisseria gonorrhoeae: a wayward pathogen: microbiological concepts, antimicrobial resistance and its epidemiological surveillance in Chile

Resumen Neisseria gonorrhoeae es un diplococo gramnegativo, no móvil, esporulado, aerobio o anaerobio facultativo, catalasa y oxidasa positivas. Las infecciones de transmisión sexual causadas por este microorganismo son un problema de salud pública definido como tal desde el siglo XIX, representando una gran amenaza para la salud humana debido a la su alta prevalencia y multirresistencia a antimicrobianos. En las últimas décadas han aumentado los reportes de cepas resistentes a penicilina, fluoroquinolonas, sulfonamidas, tetraciclina, macrólidos, y más recientemente a cefalosporinas y azitromicina. Tal panorama ha generado preocupación a nivel mundial, debido al aumento de casos de gonorrea asociados a cepas multirresistentes. En Chile se desarrolló desde el 2010 hasta el 2018 el Programa de Vigilancia de N. gonorrhoeae a nivel nacional con el objeto de caracterizar esta infección en las regiones y registrar la resistencia a los antimicrobianos. Esta revisión presenta un análisis sistemático bibliográfico, actualizado, de los principales aspectos de este microorganismo, su respuesta a antimicrobianos, y entrega pautas de diagnóstico y tratamiento, a la espera de avanzar en la comprensión del mecanismo molecular y las interacciones metabólicas e inmunológicas que determinan la infección, con miras a diseñar una vacuna efectiva.

Abstract Neisseria gonorrhoeae is a nonmotile, sporulated, aerobic or facultative anaerobic gram-negative diplococcus, catalase and oxidase positive. Sexually transmitted infections caused by this microorganism were established as public health problem since the 19th century, representing a great threat to human health due to its high prevalence and multi-resistance to antimicrobials. In recent decades, reports of strains resistant to penicillin, fluoroquinolones, sulfonamides, tetracycline, macrolides, and more recently to cephalosporins and azithromycin have increased. Such a panorama has generated concern worldwide, due to the increase in cases of gonorrhea associated with multi-resistant strains. In Chile, from 2010 to 2018, the National Surveillance Program for N. gonorrhoeae was developed in order to characterize this infection in the regions and record antimicrobial resistance. This review presents an updated, systematic bibliographic analysis of the main aspects of this microorganism, its response to antimicrobials, and provides diagnostic and treatment guidelines, while waiting to advance in the understanding of the molecular mechanism and the metabolic and immunological interactions that determine infection, with a view to designing an effective vaccine.

Kidney transplant surgical director training: Urologists represent a functional alternative to general surgeons.

INTRODUCTION: Kidney transplant (KT) directors are general surgeons or urologists. All KT centers must meet established performance standards. However, it has not been established if general surgery and urology led programs have disparate outcomes. METHODS: Transplant outcomes and donor-recipient characteristics by director training were investigated. Organ Procurement and Transplantation Network (OPTN) directory, program websites were analyzed for surgical director demographics. Scientific Registry of Transplant Recipients (SRTR) 1-year kidney survival and deceased donor (DD) wait-time rankings were evaluated. A retrospective analysis of 142 157 KT recipients from 2010 to 2019 was performed using the United Network for Organ Sharing (UNOS) database. RESULTS: One hunderd and seventy three (90.6%) KT programs were led by general surgeons. There were no significant differences in gender, ethnicity, region, credentials, or fellowship completion. Recipients undergoing KT with urology led programs were older (P = .002) and had longer wait-times (P < .001). These centers used higher KDPI (.47 vs. .45, P < .001) and higher HLA mismatch (3.92 vs. 3.89, P = .02) kidneys. Urology led centers utilized living donors less frequently (32.1% vs. 35.8%, P < .001) and had longer CIT (15.44 vs. 12.21, P < .001). Both had similar SRTR ranking of 1-year survival and DD wait-time. CONCLUSION: Most directors were general surgeon. Patient outcomes did not differ by transplant director training. Urologists represent a viable option for KT leadership and recruitment should be encouraged.

Standardising clinical outcomes measures for adult clinical trials in Fabry disease: A global Delphi consensus.

BACKGROUND: Recent years have witnessed a considerable increase in clinical trials of new investigational agents for Fabry disease (FD). Several trials investigating different agents are currently in progress; however, lack of standardisation results in challenges to interpretation and comparison. To facilitate the standardisation of investigational programs, we have developed a common framework for future clinical trials in FD. METHODS AND FINDINGS: A broad consensus regarding clinical outcomes and ways to measure them was obtained via the Delphi methodology. 35 FD clinical experts from 4 continents, representing 3389 FD patients, participated in 3 rounds of Delphi procedure. The aim was to reach a consensus regarding clinical trial design, best treatment comparator, clinical outcomes, measurement of those clinical outcomes and inclusion and exclusion criteria. Consensus results of this initiative included: the selection of the adaptative clinical trial as the ideal study design and agalsidase beta as ideal comparator treatment due to its longstanding use in FD. Renal and cardiac outcomes, such as glomerular filtration rate, proteinuria and left ventricular mass index, were prioritised, whereas neurological outcomes including cerebrovascular and white matter lesions were dismissed as a primary or secondary outcome measure. Besides, there was a consensus regarding the importance of patient-related outcomes such as general quality of life, pain, and gastrointestinal symptoms. Also, unity about lysoGb3 and Gb3 tissue deposits as useful surrogate markers of the disease was obtained. The group recognised that cardiac T1 mapping still has potential but requires further development before its widespread introduction in clinical trials. Finally, patients with end-stage renal disease or renal transplant should be excluded unless a particular group for them is created inside the clinical trial. CONCLUSION: This consensus will help to shape the future of clinical trials in FD. We note that the FDA has, coincidentally, recently published draft guidelines on clinical trials in FD and welcome this contribution.

Ophthalmological findings in patients with leukaemia in a Colombian population. / Hallazgos oftalmológicos en pacientes con leucemia en una población colombiana.

OBJECTIVE: To report the ocular manifestations in patients with leukaemia. METHODS: This is a retrospective, descriptive and observational study in patients with ocular manifestations of leukaemia. RESULTS: A total of 14 eyes were evaluated corresponding to 8 patients (5 women and 3 men) with ocular manifestations of leukaemia. The mean age at diagnosis was 43 years (31-76 years). Six eyes corresponded to patients with acute myeloid leukaemia (AML), four eyes to acute lymphoid leukaemia (ALL), two eyes to chronic myeloid leukaemia (CML), and the remaining two belonged to patients with hairy cell leukaemia (HCL). The primary ocular findings were choroidal invasion in 12 eyes (85.7%), retinal infiltration in 4 eyes (28.6%), and neuro-ophthalmic disorders in 4 eyes (28.6%). The mean visual acuity improved from 0.689 to 0.449 (logMAR) (P=.012) after the systemic and intrathecal chemotherapy. Of the eight patients, four died from systemic complications of the underlying disease. CONCLUSIONS: This is the first report of multiple ocular manifestations secondary to leukaemia in a Colombian population. It is important to keep in mind that this disease is included within the masquerade syndromes and that the ophthalmological findings that, while subtle, can also be devastating and be signs of a life-threatening disease.

Renin-angiotensin system and cancer: epidemiology, cell signaling, genetics and epigenetics.

Day by day, the health and economical burden of cancer increases globally. Indeed it can be considered that there is ''cancer pandemic''. Blocking the renin-angiotensin system (RAS) by angiotensin-converting enzyme (ACE) inhibitors (ACEI) or angiotensin-receptor blockers (ARB) are widely used measures to treat hypertension and heart failure. It has been recently suggested the activation and blocking of RAS has been associated with various types of cancer in epidemiological and experimental studies. Various studies have shown that RAS blockage is protective in some cancers. However, although fewer, contradictory data also showed that RAS blockage is either not related or adversely related to cancer. Although the reasons for these findings are not exactly known, different types of receptors and effectors in RAS may account for these findings. In the current review, we summarize the different RAS receptors and cancer development with regard to epidemiology, and pathogenesis including cell signaling pathways, apoptosis, genetic and epigenetic factors.

El ojo: Un rompecabezas diagnóstico: Estudio observacional descriptivo y revisión / The Eye: A Diagnostic Puzzle: Descriptive observational study and review

Se comunica una serie de casos, multicéntricos de la cual participaron cinco instituciones. La muestra fue de 17 pacientes, de los cuales 11 pertenecían al Hospital Dr. J.M. Cullen. Todos consultaron por compromiso orbitario y/o periorbitario. El compromiso en hombres fue de 23.4% y un 76.6% en mujeres. La edad media en años fue de 45.4 (17-69 años). Dentro de los diagnósticos encontrados, cinco casos fueron Enfermedad Relacionada con IgG4 (ER-IgG4), dos casos de Enfermedad de Erdheim Chester (EEC), dos Xantogranuloma, dos xantelasmas, un caso de metástasis de cáncer de mama, un caso de orbitopatía tiroidea, un caso de Amiloidosis con mieloma múltiple, y tres sin diagnóstico. Se revisan los diagnósticos diferenciales encontrados.

A series of multicentric cases is reported, of which five institutions participated. The sample was of 17 patients, of which 11 belonged to our Hospital, the Dr. J.M. Cullen Hospital. All consulted for orbital and/or periorbital commitment. The commitment in men was 23.4% and 76.6% in women. The average age in years was 45.4 (17-69 years). Among the diagnoses found, five cases were IgG4-Related Disease, two cases of Erdheim Chester Disease, two Xantogranuloma, two xanthelasmas, a case of breast cancer metastases, a case of thyroid orbitopathy, a case of Amyloidosis with multiple myeloma, and three without diagnosis. Differential diagnoses found are reviewed.

Viejas enfermedades conocidas que afectan la órbita: Presentación de un caso clínico y revisión / Old known diseases affecting the orbit: Presentation of a clinical case and review

Presentamos el caso de un paciente de 56 años con antecedente de Mieloma Múltiple, que evoluciona con inflamación periorbitaria de ambos parpados, bilateral, de coloración amarillenta-violácea, ulcerada e indolora. Se planteó el diagnóstico diferencial con las enfermedades xantogranulomatosas orbitarias del adulto, en especial con su asociación con Mieloma Múltiple (MM). Al realizar biopsia de la lesión, se observan en la histología fragmentación de haces de colágeno dérmico y alrededor de vasos un material amorfo acelular eosinófilo pálido, rojo congo positivo y coloración verde manzana con luz polarizada, llegando al diagnóstico de Amiloidosis y Xantogranuloma Orbitario del Adulto.

We present the case of a 56-year-old patient with a history of Multiple Myeloma, who evolves with periorbital inflammation of both eyelids, bilateral, yellowish-violet, ulcerated and painless. The differential diagnosis with the adult orbital xanthogranulomatous diseases was raised, especially XN due to its association with MM. When performing a biopsy of the lesion, fragmentation of dermal collagen bundles and around vessels a pale eosinophilic acellular material, positive congo red and apple-green color with polarized light are observed in the histology, reaching the diagnosis of Amyloidosis and Orbital Xantogranuloma of the Adult.

Pitiriasis rubra pilaris: a propósito de un caso / Pityriasis rubra pilaris: case report

RESUMEN Se presenta caso de paciente masculino de 9 años de edad, fototipo VI acude a consulta especializada de Dermatología en el Hospital pediátrico William Soler por lesiones eritematoescamosas dispuestas en placas localizadas en ambas rodillas, acompañadas de queratodermia palmo plantar. Se realizan exámenes complementarios y biopsia de piel concluyéndose caso como pitiriasis rubra pilaris circunscrita juvenil. Esta dermatosis es de infrecuente presentación, desafío terapéutico por ausencia de estandarización internacional y cursa con una evolución impredecible.

ABSTRACT We present the case of a 9-year-ol, phototype 3, who attended a specialized dermatology consultation at the William Soler pediatric hospital due to erythematosquamous lesions arranged in plaques located on both knees accompanied by palmoplantar keratoderma. Laboratory tests and skin biopsies were carried out, concluding case as a juvenile and circumscribed pityriasisrubra pilaris. This is an infrequent dermatosis, a therapeutic challenge because of the absence of international standardized treatments and it has an unpredictable evolution.

Pulse immunosuppressive therapy for multiple sclerosis during the SARS-CoV-2 lockdown de-escalation plan: Safety algorithm. / Tratamiento inmunosupresor en pulsos en esclerosis múltiple durante el desescalado de la epidemia por SARS-CoV-2. Algoritmo de seguridad.

INTRODUCTION: The COVID-19 pandemic is changing approaches to diagnosis, treatment, and care provision in multiple sclerosis (MS). During both the initial and peak phases of the epidemic, the administration of disease-modifying drugs, typically immunosuppressants administered in pulses, was suspended due to the uncertainty about their impact on SARS-CoV-2 infection, mainly in contagious asymptomatic/presymptomatic patients. The purpose of this study is to present a safety algorithm enabling patients to resume pulse immunosuppressive therapy (PIT) during the easing of lockdown measures. METHODS: We developed a safety algorithm based on our clinical experience with MS and the available published evidence; the algorithm assists in the detection of contagious asymptomatic/presymptomatic cases and of patients with mild symptoms of SARS-CoV-2 infection with a view to withdrawing PIT in these patients and preventing new infections at day hospitals. RESULTS: We developed a clinical/microbiological screening algorithm consisting of a symptom checklist, applied during a teleconsultation 48hours before the scheduled session of PIT, and PCR testing for SARS-CoV-2 in nasopharyngeal exudate 24hours before the procedure. CONCLUSION: The application of our safety algorithm presents a favourable risk-benefit ratio despite the fact that the actual proportion of asymptomatic and presymptomatic individuals is unknown. Systematic PCR testing, which provides the highest sensitivity for detecting presymptomatic cases, combined with early detection of symptoms of SARS-CoV-2 infection may reduce infections and improve detection of high-risk patients before they receive PIT.