Dexamethasone-cyclophosphamide pulse therapy outcomes comparing pemphigus vulgaris and pemphigus foliaceus groups in a Brazilian cohort study.

BACKGROUND: Dexamethasone-cyclophosphamide pulse (DCP) and dexamethasone pulse (DP) have been successfully used to treat pemphigus, but DCP/DP outcomes comparing pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are scarce. OBJECTIVE: To compare DCP/DP outcomes in a Brazilian cohort of PV and PF patients according to demographic and clinical data. METHODS: Retrospective analytical cohort study, reviewing medical charts of PV and PF patients (for DCP/DP Phases I‒IV consult Pasricha et al.16‒18). RESULTS: 37 PV and 41 PF patients non responsive to usual treatments were included similarly for DCP or DP therapy. Disease duration was longer among PF before DCP/DP prescription (p < 0.001); PF required a higher number of monthly pulses to acquire remission in Phase I (median 10 and 6 pulses, respectively; p = 0.005). DCP/DP outcomes were similar in both groups: remission in 37.8% of PV and 34.1% of PF after completed DCP/DP cycles following a median of 13 months (1-56 months follow-up); failure occurred in 13.5% of PV and 14.6% of PF in Phase I; relapse in 13.5% of PV and 12.2% of PF, and dropout in 27% of PV and 24.4% of PF in Phases II to IV. Mild side effects were documented. STUDY LIMITATIONS: The severity of PV and PF disease was not assessed by score indexes. CONCLUSIONS: PV and PF patients presented similar DCP/DP outcomes. DCP/DP should be initiated earlier in PF patients due to the longer duration of their disease in order to decrease the number of pulses and the duration of Phase I to acquire remission.

Dexamethasone-cyclophosphamide pulse therapy outcomes comparing pemphigus vulgaris and pemphigus foliaceus groups in a Brazilian cohort study

Abstract Background Dexamethasone-cyclophosphamide pulse (DCP) and dexamethasone pulse (DP) have been successfully used to treat pemphigus, but DCP/DP outcomes comparing pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are scarce. Objective To compare DCP/DP outcomes in a Brazilian cohort of PV and PF patients according to demographic and clinical data. Methods Retrospective analytical cohort study, reviewing medical charts of PV and PF patients (for DCP/DP Phases I‒IV consult Pasricha et al.16‒18). Results 37 PV and 41 PF patients non responsive to usual treatments were included similarly for DCP or DP therapy. Disease duration was longer among PF before DCP/DP prescription (p < 0.001); PF required a higher number of monthly pulses to acquire remission in Phase I (median 10 and 6 pulses, respectively; p = 0.005). DCP/DP outcomes were similar in both groups: remission in 37.8% of PV and 34.1% of PF after completed DCP/DP cycles following a median of 13 months (1-56 months follow-up); failure occurred in 13.5% of PV and 14.6% of PF in Phase I; relapse in 13.5% of PV and 12.2% of PF, and dropout in 27% of PV and 24.4% of PF in Phases II to IV. Mild side effects were documented. Study limitations The severity of PV and PF disease was not assessed by score indexes. Conclusions PV and PF patients presented similar DCP/DP outcomes. DCP/DP should be initiated earlier in PF patients due to the longer duration of their disease in order to decrease the number of pulses and the duration of Phase I to acquire remission.

Controversia justificada acerca de los esteroides particulados epidurales / A justified controversy about epidural particulate steroids

Introducción: La administración epidural de esteroides constituye un pilar del tratamiento del dolor radicular cervical y lumbosacro. Objetivo: Describir los mecanismos fisiológicos y características farmacológicas de los corticosteroides utilizados en el tratamiento del dolor, así como las complicaciones derivadas de la administración epidural de esteroides particulados. Métodos: Se realizó una revisión no sistemática de la literatura en bases de datos científicas como Cochrane Database of Systematic Reviews, Pubmed/Medline, EMBASE, Scopus, Web of Science, EBSCOhost, ScienceDirect, OVID y el buscador académico Google Scholar, en los meses de julio a septiembre del año 2020. Desarrollo: Los corticoides utilizados en la medicina del dolor son derivados de la prednisolona. Estos se clasifican en particulados (de depósito, de suspensión) o no particulados (de dilución), en función de la presencia o ausencia de un componente molecular sólido (moléculas tipo éster, insolubles en agua). Los fármacos más empleados son la dexametasona, betametasona, triamcinolona y metilprednisolona. Conclusiones: La administración epidural de esteroides particulados está relacionada con la incidencia de complicaciones graves, aunque poco frecuentes, como paraplejía, tetraplejía, infarto de la médula espinal, hemorragia y edema cerebral. La evidencia disponible muestra una efectividad analgésica similar a los compuestos no particulados. Por lo tanto, no se recomienda su utilización rutinaria durante el abordaje del espacio epidural(AU)

Introduction: Epidural administration of steroids is a cornerstone for the treatment of cervical and lumbosacral radicular pain. Objective: To describe the physiological mechanisms and pharmacological characteristics of the corticosteroids used for pain treatment, as well as the complications derived from the epidural administration of particulate steroids. Methods: A nonsystematic review of the literature was carried out, from July to September 2020, in scientific databases such as Cochrane Database of Systematic Reviews, Pubmed/Medline, EMBASE, Scopus, Web of Science, EBSCOhost, ScienceDirect, OVID and the academic search engine Google Scholar. Development: The corticoids used in pain medicine are derived from prednisolone. These are classified into particulate (deposit, suspension) or non-particulate (dilution), depending on the presence or absence of a solid molecular component (ester-type molecules, insoluble in water). The most commonly used drugs are dexamethasone, betamethasone, triamcinolone, and methylprednisolone. Conclusions: The epidural administration of particulate steroids is related to the incidence of serious, although infrequent, complications, such as paraplegia, tetraplegia, spinal cord infarction, hemorrhage and cerebral edema. Available evidence shows analgesic effectiveness similar to that of non-particulate compounds. Therefore, its routine usage is not recommended during the managment of the epidural space(AU)

Demodicose felina em Santa Catarina, Brasil / Feline demodicosis in Santa Catarina state, Brazil

A demodicose felina é considerada uma dermatopatia rara e pode ser causada pelos ácaros Demodex cati, Demodex gatoi e uma terceira espécie ainda não nomeada. Foi atendido um felino adulto apresentando prurido intenso há 9 meses e histórico de tratamento com cefalexina e prednisolona, com piora progressiva. Ao exame físico, havia alopecia, hiperqueratose, escoriações e eritema em cabeça, pescoço, região lombossacra, cauda e membros pélvicos, além da presença de pulgas. Para puliciose, foram prescritos selamectina spot on a cada 30 dias e uso de amitraz no ambiente a cada sete dias e, para controle da infecção secundária pelas escoriações, foram recomendados banhos semanais com clorexidine. Realizaram-se raspado de pele profundo e arrancamento de pelos para tricograma e exame parasitológico de pele, respectivamente, com diagnósticos de demodicose por Demodex cati, e dermatite micótica associada a infecção bacteriana secundária. O tratamento foi modificado para uso de selamectina a cada 2 semanas, mas tutor não retornou e informou, após vários meses, ter feito terapia com selamectina apenas a cada 30 dias e descontinuidade dos banhos. Não foi possível associar a demodicose, para este felino, a outras comorbidades e acredita-se que a apresentação generalizada da doença tenha se dado pelo prurido causado pela puliciose.

Feline demodicosis is considered a rare dermatopathy and can be caused by Demodex cati, Demodex gatoi and a third species not yet named. An adult male feline was attended with severe pruritus for 9 months and a history of treatment with cephalexin and prednisolone, with progressive worsening. On physical examination, there was alopecia, hyperkeratosis, abrasions and erythema on the head, neck, lumbosacral region, tail and pelvic limbs, in addition to the presence of fleas. For pulicosis, selamectin spot on was prescribed every 30 days and use of amitraz in the environment every seven days. In order to control secondary infection, weekly baths with chlorhexidine were recommended. Deep skin scraping and hair plucking were performed for trichogram and parasitological skin examination, respectively, with diagnoses of demodicosis by Demodex cati, and mycotic dermatitis associated with secondary bacterial infection. The treatment was modified to use selamectin every 2 weeks, but the tutor did not return and reported, after several months, that he had done therapy with selamectin only every 30 days and discontinued baths. For this feline, it was not possible to associate demodicosis with other comorbidities. It is believed that the generalized presentation of the disease occurred due to the pruritus caused by pulicosis.

Feline demodicosis in Santa Catarina state, Brazil / Demodicose felina em Santa Catarina, Brasil

Feline demodicosis is considered a rare dermatopathy and can be caused by Demodex cati, Demodex gatoi and a third species not yet named. An adult male feline was attended with severe pruritus for 9 months and a history of treatment with cephalexin and prednisolone, with progressive worsening. On physical examination, there was alopecia, hyperkeratosis, abrasions and erythema on the head, neck, lumbosacral region, tail and pelvic limbs, in addition to the presence of fleas. For pulicosis, selamectin spot on was prescribed every 30 days and use of amitraz in the environment every seven days. In order to control secondary infection, weekly baths with chlorhexidine were recommended. Deep skin scraping and hair plucking were performed for trichogram and parasitological skin examination, respectively, with diagnoses of demodicosis by Demodex cati, and mycotic dermatitis associated with secondary bacterial infection. The treatment was modified to use selamectin every 2 weeks, but the tutor did not return and reported, after several months, that he had done therapy with selamectin only every 30 days and discontinued baths. For this feline, it was not possible to associate demodicosis with other comorbidities. It is believed that the generalized presentation of the disease occurred due to the pruritus caused by pulicosis.

A demodicose felina é considerada uma dermatopatia rara e pode ser causada pelos ácaros Demodex cati,Demodex gatoi e uma terceira espécie ainda não nomeada. Foi atendido um felino adulto apresentando prurido intenso há 9 meses e histórico de tratamento com cefalexina e prednisolona, com piora progressiva. Ao exame físico, havia alopecia, hiperqueratose, escoriações e eritema em cabeça, pescoço, região lombossacra, cauda e membros pélvicos, além da presença de pulgas. Para puliciose, foram prescritos selamectina spot on a cada 30 dias e uso de amitraz no ambiente a cada sete dias e, para controle da infecção secundária pelas escoriações, foram recomendados banhos semanais com clorexidine. Realizaram-se raspado de pele profundo e arrancamento de pelos para tricograma e exame parasitológico de pele, respectivamente, com diagnósticos de demodicose por Demodex cati, e dermatite micótica associada a infecção bacteriana secundária. O tratamento foi modificado para uso de selamectina a cada 2 semanas, mas tutor não retornou e informou, após vários meses, ter feito terapia com selamectina apenas a cada 30 dias e descontinuidade dos banhos. Não foi possível associar a demodicose, para este felino, a outras comorbidades e acredita-se que a apresentação generalizada da doença tenha se dado pelo prurido causado pela puliciose.

Decreased Enteric Bacterial Composition and Diversity in South American Crohn's Disease Vary With the Choice of Treatment Strategy and Time Since Diagnosis.

BACKGROUND AND AIMS: The symptomology of Crohn's disease [CD], a chronic inflammatory disease of the digestive tract, correlates poorly with clinical, endoscopic or immunological assessments of disease severity. The prevalence of CD in South America is rising, reflecting changes in socio-economic stability. Many treatment options are available to CD patients, including biological agents and corticosteroids, each of which offers variable efficacy attributed to host genetics and environmental factors associated with alterations in the gut microbiota. METHODS: Based on 16S rRNA gene sequencing and taxonomic differences, we compared the faecal microbial population of Brazilian patients with CD treated with corticosteroid or anti-tumour necrosis factor [anti-TNF] immunotherapy. Faecal calprotectin and plasma sCD14 levels were quantified as markers for local and systemic inflammation, respectively. RESULTS: Anti-TNF treatment led to an increased relative abundance of Proteobacteria and a decreased level of Bacteroidetes. In contrast, corticoid treatment was associated with an increase in the relative abundance of Actinobacteria, which has been linked to inflammation in CD. Disruption of the faecal microbiota was related to decreased bacterial diversity and composition. Moreover, the choice of clinical regimen and time since diagnosis modulate the character of the resulting dysbiosis. CONCLUSIONS: Enteric microbial populations in CD patients who have been treated are modulated by disease pathogenesis, local inflammatory microenvironment and treatment strategy. The dysbiosis that remains after anti-TNF treatment due to decreased bacterial diversity and composition abates restoration of the microbiota to a healthy state, suggesting that the identification and development of new clinical treatments for CD must include their capacity to normalize the gut microbiota.

Demodicose felina em Santa Catarina, Brasil / Feline demodicosis in Santa Catarina state, Brazil

A demodicose felina é considerada uma dermatopatia rara e pode ser causada pelos ácaros Demodex cati, Demodex gatoi e uma terceira espécie ainda não nomeada. Foi atendido um felino adulto apresentando prurido intenso há 9 meses e histórico de tratamento com cefalexina e prednisolona, com piora progressiva. Ao exame físico, havia alopecia, hiperqueratose, escoriações e eritema em cabeça, pescoço, região lombossacra, cauda e membros pélvicos, além da presença de pulgas. Para puliciose, foram prescritos selamectina spot on a cada 30 dias e uso de amitraz no ambiente a cada sete dias e, para controle da infecção secundária pelas escoriações, foram recomendados banhos semanais com clorexidine. Realizaram-se raspado de pele profundo e arrancamento de pelos para tricograma e exame parasitológico de pele, respectivamente, com diagnósticos de demodicose por Demodex cati, e dermatite micótica associada a infecção bacteriana secundária. O tratamento foi modificado para uso de selamectina a cada 2 semanas, mas tutor não retornou e informou, após vários meses, ter feito terapia com selamectina apenas a cada 30 dias e descontinuidade dos banhos. Não foi possível associar a demodicose, para este felino, a outras comorbidades e acredita-se que a apresentação generalizada da doença tenha se dado pelo prurido causado pela puliciose.(AU)

Feline demodicosis is considered a rare dermatopathy and can be caused by Demodex cati, Demodex gatoi and a third species not yet named. An adult male feline was attended with severe pruritus for 9 months and a history of treatment with cephalexin and prednisolone, with progressive worsening. On physical examination, there was alopecia, hyperkeratosis, abrasions and erythema on the head, neck, lumbosacral region, tail and pelvic limbs, in addition to the presence of fleas. For pulicosis, selamectin spot on was prescribed every 30 days and use of amitraz in the environment every seven days. In order to control secondary infection, weekly baths with chlorhexidine were recommended. Deep skin scraping and hair plucking were performed for trichogram and parasitological skin examination, respectively, with diagnoses of demodicosis by Demodex cati, and mycotic dermatitis associated with secondary bacterial infection. The treatment was modified to use selamectin every 2 weeks, but the tutor did not return and reported, after several months, that he had done therapy with selamectin only every 30 days and discontinued baths. For this feline, it was not possible to associate demodicosis with other comorbidities. It is believed that the generalized presentation of the disease occurred due to the pruritus caused by pulicosis.(AU)

Breast cancer and pyoderma gangrenosum: a complication after conservative surgery and radiotherapy

Pyoderma gangrenosum (PG) is a rare, ulcerative, and painful neutrophilic dermatosis of unknown cause associated with systemic diseases and/or pathergy phenomenon in 30% of cases. We report the case of a breast cancer patient submitted to oncoplastic conservative surgery followed by adjuvant radiotherapy, with long-term progression to PG. It's rare and challeng ing nature reinforces the need for early diagnosis to increase treatment effectiveness and reduce morbidity.

A Single Dose of Methylprednisolone Improves Aversive Memory Consolidation and Extinction in Rats.

Aversive memory is essential for survival, but in some situations its exacerbation can be potentially dangerous. There are several ways to modulate memory, among them, through stress-related hormones physiological release or administration of exogenous substances analogous to them. Recently, our group shown that a chronic treatment with a low dose of methylprednisolone (MP) is able to promote memory persistence in rats. Herein, we evaluate if a single intraperitoneal (IP) dose of MP (5 mg/kg) is able to modulate aversive memory consolidation and promote memory persistence and extinction in rats. For this, two experiments were carried out. In the first one, we demonstrated that a single IP MP administration in specific times after inhibitory avoidance (IA) training improved memory consolidation and persistence. In the second experiment, we verified that a single IP MP administration 2 h after IA extinction training promoted memory extinction. This results suggest a possible new clinical applicability for MP on the aversive memory disorders, as post-traumatic stress.

Glomerulonefritis rápidamente progresiva de etiología posinfecciosa. Caso pediátrico / Postinfectious rapidly progressive glomerulonephritis in a pediatric patient

La glomerulonefritis rápidamente progresiva de etiología posinfecciosa es rara en la infancia, con una prevalencia estimada del 1-3 %. La mayoría debuta como insuficiencia renal aguda y su tratamiento se basa en el uso de corticoides y ciclofosfamida. Si se realiza diagnóstico precoz, el 70 % presenta una recuperación temprana de la función renal. En los últimos años, se han descrito "glomerulopatías por C3", que presentan características que se superponen. Son útiles, en el diagnóstico diferencial, la inmunofluorescencia y la determinación del factor nefrítico. Se presenta un varón de 4 años que acude por fiebre y cuadro respiratorio. Se observa microhematuria, proteinuria, descenso de filtrado glomerular y descenso de C3, y se sospecha glomerulonefritis aguda. Se realiza una biopsia, cuya microscopía óptica muestra la presencia de semilunas epiteliales, y la electrónica, depósitos subepiteliales en forma de joroba, por lo que se diagnostica glomerulonefritis rápidamente progresiva de etiología posinfecciosa.

Postinfectious glomerulonephritis is rarely presented as rapidly progressive glomerulonephritis in children; the prevalence is approximately 1-3 %. Most children have acute onset of renal failure; initial treatment involves corticosteroids and immunosuppressive therapy. Early diagnosis improves prognosis. In recent years, an entity known as "C3 glomerulopathies" has been described, presenting characteristics that overlap. In the differential diagnosis, the immunofluorescence and the determination of the nephritic factor are useful. We report a 4-year-old boy with fever, respiratory symptoms and hyporexia. Microhematuria, proteinuria, decline in glomerular filtration and depressed C3 were found. Acute glomerulonephritis was suspected. Renal biopsy showed crescent formation, immunofluorescence staining for C3 and subepithelial humps. Therefore, postinfectious glomerulonephritis with crescent formations was diagnosed.

[Postinfectious rapidly progressive glomerulonephritis in a pediatric patient]. / Glomerulonefritis rápidamente progresiva de etiología posinfecciosa. Caso pediátrico.

Postinfectious glomerulonephritis is rarely presented as rapidly progressive glomerulonephritis in children; the prevalence is approximately 1-3 %. Most children have acute onset of renal failure; initial treatment involves corticosteroids and immunosuppressive therapy. Early diagnosis improves prognosis. In recent years, an entity known as "C3 glomerulopathies" has been described, presenting characteristics that overlap. In the differential diagnosis, the immunofluorescence and the determination of the nephritic factor are useful. We report a 4-year-old boy with fever, respiratory symptoms and hyporexia. Microhematuria, proteinuria, decline in glomerular filtration and depressed C3 were found. Acute glomerulonephritis was suspected. Renal biopsy showed crescent formation, immunofluorescence staining for C3 and subepithelial humps. Therefore, postinfectious glomerulonephritis with crescent formations was diagnosed.

La glomerulonefritis rápidamente progresiva de etiología posinfecciosa es rara en la infancia, con una prevalencia estimada del 1-3 %. La mayoría debuta como insuficiencia renal aguda y su tratamiento se basa en el uso de corticoides y ciclofosfamida. Si se realiza diagnóstico precoz, el 70 % presenta una recuperación temprana de la función renal. En los últimos años, se han descrito "glomerulopatías por C3", que presentan características que se superponen. Son útiles, en el diagnóstico diferencial, la inmunofluorescencia y la determinación del factor nefrítico. Se presenta un varón de 4 años que acude por fiebre y cuadro respiratorio. Se observa microhematuria, proteinuria, descenso de filtrado glomerular y descenso de C3, y se sospecha glomerulonefritis aguda. Se realiza una biopsia, cuya microscopía óptica muestra la presencia de semilunas epiteliales, y la electrónica, depósitos subepiteliales en forma de joroba, por lo que se diagnostica glomerulonefritis rápidamente progresiva de etiología posinfecciosa.

Polineuropatía desmielinizante inflamatoria crónica: revisión a propósito de un caso / Chronic inflammatory demyelinating polyneuropathy: review, case and report

Resumen La polineuropatía desmielinizante inflamatoria crónica (CIDP por sus siglas en inglés) corresponde a un espectro de diferentes fenotipos clínicos caracterizados por lesiones de naturaleza autoinmune, inflamatoria y desmielinizante, que afectan primariamente nervios periféricos y raíces nerviosas. Generalmente, los pacientes con CIDP presentan un curso crónico de discapacidad neurológica, pero hasta un tercio de los casos puede exhibir un curso remitente-recidivante. El fenotipo clásico involucra compromiso simétrico de la fuerza muscular y la sensibilidad proximal y distal, asociado a arreflexia generalizada. El diagnóstico requiere la demostración de la desmielinización de nervios mediante electromiografía o biopsia de nervios. Debido a la afectación de personas relativamente jóvenes, laboralmente activos, y a la gran discapacidad neurológica que puede generar, el tratamiento debiera ser iniciado precozmente. Los pilares de la terapia en su fase inicial son los corticoides intravenosos en altas dosis, inmunoglobulina intravenosa y la plasmaféresis, mientras que la terapia de mantención se basa, principalmente, en el uso de corticoides orales a bajas dosis. Este artículo presenta el caso de un paciente evaluado en nuestro hospital y diagnosticado con CIDP, y expone una revisión bibliográfica actualizada de la enfermedad.

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) can be defined as a spectrum of different clinical phenotypes which are characterized by autoimmune, inflammatory and demyelinating injuries, primarily affecting the peripheral nerves and nerve roots. Most patients with CIDP have a chronic course of neurological disability, but about a third of cases exhibit a relapsing-remitting course. Classic phenotype of CIDP involves symmetric compromise of proximal and distal muscle strength and sensitivity, associated with generalized areflexia. For an accurate diagnosis, demonstration of nerve demyelination by electromyography or nerve biopsy is required. Due to the affectation of relatively young, labor-active people and the high risk for neurological disability by the disease, treatment should be initiated early. The predominant lines of therapy, in its initial phase, are high-dose intravenous corticosteroids, intravenous immunoglobulin and plasmapheresis, while the maintenance therapy is mainly based on low-dose oral corticosteroids. This article presents a case report of a patient evaluated in our hospital and diagnosed with CIDP and exposes an updated literature review about this disease.

[Peripheral nervous system immunological disorder]. / Enfermedades inmunomediadas del sistema nervioso periférico.

Autoimmune diseases of the peripheral nervous system are common in pediatrics. Guillain-Barré syndrome, juvenile myasthenia gravis, and juvenile dermatomyositis are the most important. Their common pathogenesis involves the action of specific autoantibodies which are frequently triggered by viral or bacterial infection. Acute inflammatory demyelinating polyneuropathy is the most frequent pathological feature. There is also a motor axonal form. Both have a progressive ascending clinical course. The specific treatment is immunoglobulin 2 g/kg. Juvenile myasthenia gravis is expressed by ocular signs and generalized and fluctuating fatigability. It can involve respiratory functions triggering a myasthenic crisis. It is treated with anticholinesterase agents, corticosteroids, immunoglobulins, and immunosuppressants. Thymectomy has recently shown effectiveness. Juvenile dermatomyositis is expressed by skin and muscle signs. Elevated muscle enzymes, muscle biopsy, and magnetic resonance imaging contribute to the diagnosis. It is treated with corticosteroids, immunoglobulins, and immunosuppressants. All three disorders, Guillain-Barré, juvenile myasthenia gravis, and juvenile dermatomyositis have a good prognosis.

Enfermedades inmunomediadas del sistema nervioso periférico / Peripheral nervous system immunological disorder

Las enfermedades autoinmunes del sistema nervioso periférico son frecuentes en pediatría. Las más importantes son el síndrome de Guillain-Barré, la miastenia gravis juvenil y la dermatomiositis juvenil. Tienen en común ser causadas por acción de anticuerpos específicos que producen la signología clínica, reacción que puede ser gatillada por un cuadro viral o bacteriano, como ocurre principalmente en SGB. La polineuropatía aguda inflamatoria desmielinizante es más frecuente. Existe también la forma axonal motora. Ambas tienen clínica progresiva ascendente. El tratamiento específico es la inmunoglobulina 2 g/ kg. La miastenia gravis juvenil se expresa por signos oculares, generalizados y fatigabilidad fluctuante. Puede comprometer la función respiratoria desencadenando crisis miasténica. Se trata con anticolinesterásicos, corticoides, inmunoglobulinas e inmunosupresores. La timectomía ha mostrado recientemente su efectividad. La dermatomiositis juvenil se expresa por signos cutáneos y musculares. Se diagnostica por elevación de enzimas musculares, biopsia y resonancia musculares y se trata con corticoides, inmunoglobulinas e inmunosupresores. Tanto el síndrome de Guiilain-Barré, como la miastenia gravis y la dermatomiositis juvenil, tienen buen pronóstico.

Autoimmune diseases of the peripheral nervous system are common in pediatrics. Guillain-Barré syndrome, juvenile myasthenia gravis, and juvenile dermatomyositis are the most important. Their common pathogenesis involves the action of specific autoantibodies which are frequently triggered by viral or bacterial infection. Acute inflammatory demyelinating polyneuropathy is the most frequent pathological feature. There is also a motor axonal form. Both have a progressive ascending clinical course. The specific treatment is immunoglobulin 2 g/kg. Juvenile myasthenia gravis is expressed by ocular signs and generalized and fluctuating fatigability. It can involve respiratory functions triggering a myasthenic crisis. It is treated with anticholinesterase agents, corticosteroids, immunoglobulins, and immunosuppressants. Thymectomy has recently shown effectiveness. Juvenile dermatomyositis is expressed by skin and muscle signs. Elevated muscle enzymes, muscle biopsy, and magnetic resonance imaging contribute to the diagnosis. It is treated with corticosteroids, immunoglobulins, and immunosuppressants. All three disorders, Guillain-Barré, juvenile myasthenia gravis, and juvenile dermatomyositis have a good prognosis.

Estudio Descriptivo: Variación del Índice de Tiempo de Aceleración/Tiempo de Eyección Sistólico del Tronco de la Arteria Pulmonar por Efecto de Maduración Pulmonar con Corticoides en Fetos Prematuros. Clínica Humanitaria. Cuenca ­ Ecuador, 2016 / Descriptive Research: Variation of Acceleration Time/Ejection Time Index of the Trunk of the Pulmonary Arteries Due to the Lung Maturation Effect of Corticosteroids on Preterm Fetus. Clínica Humanitaria, 2016

INTRODUCCIÓN: El nacimiento prematuro representa un problema de salud pública importante a nivel mundial y en especial en países en vías de desarrollo. En el Ecuador, en el año 2014, la tasa de mortalidad infantil fue de 8.35 defunciones infantiles por cada 1 000 nacimientos y las principales causas de mortalidad infantil fueron la dificultad respiratoria del recién nacido y los trastornos con duración corta de la gestación. MÉTODOS: El presente es un estudio observacional, descriptivo y longitudinal, realizado con una muestra censal o por conveniencia de 36 mujeres que cursaron entre 24 y 34 semanas de gestación con feto único vivo, riesgo de parto pretérmino e indicación de maduración pulmonar con corticoides. Se recogió información de flujometría fetal para determinar el efecto del uso de corticoides para maduración pulmonar. Se realizaron pruebas de normalidad Shapiro-Wilk en los resultados obtenidos antes y después de la maduración pulmonar. Para el procesamiento de los datos se utilizaron los programas Microsoft Excel® y SPSS® v.20. RESULTADOS: La edad media de este grupo fue de 25.64 años, las media de semanas de gestación fue de 31.2. La patología más frecuente fue amenaza de parto pretérmino con un 72 %, seguida de ruptura prematura pretérmino de membranas con un 13.9 %. Existió una diferencia estadísticamente significativa entre los valores de los índices Tiempo de Acelereción / Tiempo de Eyección antes y después de la maduración pulmonar (0.272 ms y 0.310 ms, respectivamente; P < 0.0001). CONCLUSIONES: La comparación de las medias de los índices Tiempo de Acelereción / Tiempo de Eyección del tronco de la arteria pulmonar pre y post maduración pulmonar con corticoides evidenció una diferencia significativa importante. Los hallazgos sugieren que la maduración pulmonar con corticoides es eficaz para mejorar el flujo de arteria pulmonar en fetos prematuros.

BACKGROUND: Premature birth represents an important public health problem worldwide and especially in developing countries. In Ecuador (2014), the infant mortality rate reached 8.35 deaths per 1 000 births and their main causes were newborns respiratory distress and gestation period short-term disorders. METHODS: This is an observational longitudinal descriptive research; it was performed in a convenience sample that included 26 singlet on pregnant women between 24 and 34 weeks of gestation. They also were on risk of preterm delivery and had indication of lung maturation with corticosteroids. Information of the fetal pulmonary arteries flow was collected to determine the effect of corticosteroids in pulmonary maturation. RESULTS: Mean age was 25.64 years and had an average gestation of 31.2 weeks. Most common diseases were preterm labor threat (72 %) and premature rupture of membranes (13.9 %). There was a statistically significant difference between the values of Acceleration Time / Ejection Time indexes before and after lung maturation (0.272 ms vs. 0.310 ms respectively; P < 0.0001). CONCLUSIONS: Comparison of Acceleration Time / Ejection Time indexes from the trunk of the pulmonary arteries before and after lung maturation evidenced an important significant difference. The findings suggest that pulmonary maturation with corticosteroids is effective to improve the pulmonary arteries flow of preterm fetus.

Síndrome de Stevens-Johnson en edad pediátrica / Stevens Johnson Syndrome of a Pediatric Age Case

El síndrome de Stevens-Johnson (SSJ) es una enfermedad inflamatoria aguda, originada por una reacción de hipersensibilidad, secundaria a ingesta de medicamentos o infecciones, que afecta a la piel y las membranas mucosas produciendo lesiones características del síndrome, causadas por apoptosis y posterior necrosis de los queratinocitos; su forma más severa es la necrolisis epidérmica tóxica, que constituye junto al SSJ un espectro de la misma enfermedad, compartiendo aspectos etiológicos, patogenéticos, histológicos y terapéuticos que ponen en peligro la vida del paciente. La afección se caracteriza por una súbita erupción morfológicamente variable, acompañada de estomatitis y oftalmia.En el presente trabajo se presenta el caso de un niño de 8 años de edad,con diagnóstico clínico de síndrome de Stevens-Johnson, con manifestaciones cutáneas, oculares y de la mucosa oral, que iniciaron posterior a ingesta de ibuprofeno, se mantuvo con un protocolo de cuidados que incluyeron soporte de oxígeno, antibioticoterapia, analgesia, corticoides, nebulizaciones, limpieza de lesiones con solución salina, sin debridación y lubricante oftálmico; tras 8 días dehospitalización el paciente evoluciona satisfactoriamente sin complicaciones durante su estancia hospitalaria.

The Stevens-Johnson syndrome (SJS) is an acute inflammatory disease caused by a hypersensitivity reaction, secondary to medication intake or infections, that affects skin and mucous membranes producing characteristic wounds of the syndrome, caused by apoptosis and subsequent necrosis of keratinocytes; the major form of this disease, is toxic epidermal necrolysis, wich together with SJS is a spectrum of the same disease, sharing etiological pathogenetic, histological and therapeutic aspects, that endanger the patient's life. The affection is characterized by a sudden morphologically varying rash, accompanied bystomatitis and ophthalmic injure. In this work we show an 8 year old patient with a clinical diagnosis of Stevens-Johnson syndrome, involving skin, eye and oral mucosa manifestations, which began after the intake of ibuprofen , it was mantained with a protocol care based on oxygen support, antibiotic therapy, analgesia, corticosteroids, sprays, cleansing wounds with saline solution without debridement and ophthalmic lubricant, after 8 days of hospitalization, our patient has a satisfactory evolution without acute complications during their time at the hospital.

Topical Dexamethasone Administration Impairs Protein Synthesis and Neuronal Regeneration in the Olfactory Epithelium.

Chronic inflammatory process in the nasal mucosa is correlated with poor smell perception. Over-activation of immune cells in the olfactory epithelium (OE) is generally associated with loss of olfactory function, and topical steroidal anti-inflammatory drugs have been largely used for treating such condition. Whether this therapeutic strategy could directly affect the regenerative process in the OE remains unclear. In this study, we show that nasal topical application of dexamethasone (DEX; 200 or 800 ng/nostril), a potent synthetic anti-inflammatory steroid, attenuates OE lesion caused by Gram-negative bacteria lipopolysaccharide (LPS) intranasal infusion. In contrast, repeated DEX (400 ng/nostril) local application after lesion establishment limited the regeneration of olfactory sensory neurons after injury promoted by LPS or methimazole. Remarkably, DEX effects were observed when the drug was infused as 3 consecutive days regimen. The anti-inflammatory drug does not induce OE progenitor cell death, however, disturbance in mammalian target of rapamycin downstream signaling pathway and impairment of protein synthesis were observed during the course of DEX treatment. In addition, in vitro studies conducted with OE neurospheres in the absence of an inflammatory environment showed that glucocorticoid receptor engagement directly reduces OE progenitor cells proliferation. Our results suggest that DEX can interfere with the intrinsic regenerative cellular mechanisms of the OE, raising concerns on the use of topical anti-inflammatory steroids as a risk factor for progressive olfactory function impairment.

Hipomanía y corticoides en pacientes oncológicos. Reporte de un caso y revisión de la literatura / Hypomania and corticoids in oncological patients. Case report and literature review

Resumen Introducción: Cada año aumenta el número de personas que padece algún tipo de cáncer. Los corticoides son ampliamente utilizados en el tratamiento de los pacientes con cáncer y su uso no está exento de efectos adversos, muchas veces muy invalidantes. Objetivo: Presentar un caso clínico de un brote hipomaníaco en una paciente con cáncer de ovario usuaria de corticoides y realizar una revisión de la literatura del tema. Caso clínico: Paciente, portadora de un cáncer de ovario avanzado, tratado con cirugía y quimioterapia, debuta con episodios de vómitos que son manejados con dexametasona. Durante el tratamiento presenta insomnio, verborrea e ideas de grandiosidad, diagnosticándose un brote hipomaniaco secundario al tratamiento esteroidal. Discusión: En el caso de los pacientes oncológicos, e independientemente del uso de corticoides, aproximadamente el 50% de ellos presentará algún tipo de sintomatologia psiquiátrica, ahora bien, si a esto agregamos el uso de corticoides la incidencia puede aumentar hasta un 65% - 75%. La hipomanía da cuenta prácticamente del 50% de los trastornos psiquiátricos inducidos por corticoides. Con dosis menores de 40 mg/día sólo el 2% de los pacientes se verá afectado por este tipo de trastornos, mientras que con dosis entre 40 a 80 mg/día esta incidencia aumenta hasta un 5%. Conclusión: Los corticoides son ampliamente utilizados en pacientes oncológicos, sin embargo, su uso puede provocar trastornos psiquiátricos. Es importante dar a conocer más al equipo sanitario referente a la asociación de corticoides y cuadros psiquiátricos para su rápida detección y manejo clínico.

Introduction: Every year the number of people affecting by cancer increase. Corticosteroids are widely used in the treatment of patients with cancer and their use is not without adverse effects, often very disabling. Objective: Present a clinical case of hypomanic outbreak in a patient with ovarian cancer using corticosteroids and to carry out a review of the literature on the subject. Clinical case: Patient with an advanced ovarian cancer, treated with surgery and chemotherapy, debuts with episodes of vomiting that are managed with dexamethasone. During the treatment he presented insomnia, verbiage and grandiosity, diagnosing a hypomanic outbreak secondary to steroidal treatment. Discussion: In cancer patients, and independently of the use of corticosteroids, approximately 50% of them will present some type psychiatric disorder. With corticosteroids the incidence can increase up to 65%-75%. Hypomania accounts for almost 50% of psychiatric disorders induced by corticosteroids. With doses lower than 40 mg/day only 2% of patients will be affected by this type of disorders, while with doses between 40 to 80 mg/day this incidence increases up to 5%. Conclusion: Corticosteroids are widely used in cancer patients, however their use can cause psychiatric disorders. It is important to make the healthcare team more aware of the association of corticosteroids and psychiatric symptoms for rapid detection and clinical management.

Dexamethasone for Parapneumonic Pleural Effusion: A Randomized, Double-Blind, Clinical Trial.

OBJECTIVE: To assess whether dexamethasone (DXM) decreases the time to recovery in patients with parapneumonic pleural effusion. STUDY DESIGN: This was a multicenter, randomized, double blind, parallel-group, placebo-controlled clinical trial of 60 children, ranging in age from 1 month to 14 years, with community-acquired pneumonia (CAP) and pleural effusion. Patients received either intravenous DXM (0.25?mg/kg/dose) or placebo every 6 hours over a period of 48 hours, along with antibiotics. The primary endpoint was the time to recovery in hours, defined objectively. We also evaluated complications and adverse events. RESULTS: Among the 60 randomized patients (mean age, 4.7 years; 58% female), 57 (95%) completed the study. Compared with placebo recipients, the patients receiving DXM had a shorter time to recovery, after adjustment by severity group and stratification by center (hazard ratio, 1.95; 95% CI, 1.10-3.45; P?=?.021). The median time to recovery for patients receiving DXM was 68 hours (2.8 days) shorter than patients receiving placebo (109 hours vs 177 hours; P?=?.037). In exploratory subgroup analysis, the median time to recovery for patients with simple effusion receiving DXM was 76 hours (3.1 days) shorter than for patients with simple effusion receiving placebo (P?=?.017). The median time to recovery for patients with complicated effusion receiving DXM was 14 hours (0.5 days) shorter than for patients with complicated effusion receiving placebo (P?=?.66). The difference in the effect of DXM in the 2 severity groups was not statistically significant (P?=?.138 for interaction). There were no significant differences in complications or adverse events attributable to the study drugs, except for hyperglycemia. CONCLUSION: In this trial, DXM seemed to be a safe and effective adjunctive therapy for parapneumonic pleural effusion. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01261546.

Effect of methylprednisolone use on the rotator cuff in rats: biomechanical and histological study.

OBJECTIVE: To evaluate the influence of treatment with different doses of methylprednisolone on the mechanical resistance and possible histological alterations of the rotator cuff tendon in rats. METHODS: Male Wistar rats were divided randomly into four treatment groups: sham, vehicle or 0.6 mg/kg or 6.0 mg/kg of methylprednisolone. Changes to mechanical resistance (in N) and histological parameters (fibrillar appearance, presence of collagen, edema and vascular proliferation) of the rotator cuff tendon were evaluated. The analyses were conducted after administration of one treatment (24 h afterwards), two treatments (7 days afterward) or three treatments (14 days afterwards), into the subacromial space. RESULTS: Seven and fourteen days after the treatments were started, it was found that in a dose-dependent manner, methylprednisolone reduced the mechanical resistance of the rotator cuff tendon (p < 0.05 in relation to the vehicle group). Modifications to the histological parameters were observed on the 7th and 14th days after the first infiltration, especially regarding the presence of collagen and vascular proliferation, for the dose of 0.6 mg/kg of methylprednisolone, and also regarding the presence of collagen, edema and vascular proliferation for the dose of 6.0 mg/kg of corticoid. CONCLUSION: The results obtained demonstrated a relationship between methylprednisolone use through infiltration into the subacromial space and reduction of the mechanical resistance of and histological modifications to the rotator cuff tendon in rats.

OBJETIVO: Avaliar a influência do tratamento com diferentes doses de metilprednisolona sobre a resistência mecânica, bem como possíveis alterações histológicas do tendão do manguito rotador (MR) em ratos. MÉTODOS: Ratos Wistar machos foram divididos aleatoriamente em quatro grupos de tratamento como sham, veículo, 0,6 mg/kg ou 6 mg/kg de metilprednisolona. Alterações na resistência mecânica (em N) e em parâmetros histológicos (aparência fibrilar, presença de colágeno, edema e proliferação vascular) do tendão do manguito rotador (MR) foram avaliadas. As análises foram feitas após o tratamento com uma (24 horas após), duas (sete dias após) ou três (14 dias após) administrações no espaço subacromial. RESULTADOS: Após sete e 14 dias do início do tratamento a metilprednisolona reduziu, de maneira dependente de dose, a resistência mecânica do tendão do MR (p < 0,05 em relação ao grupo veículo). Também foram observadas modificação em parâmetros histológicos nos dias sete e 14 após a primeira infiltração, principalmente quanto à presença de colágeno e proliferação vascular para a dose de 0,6 MG/kg de metilprednisolona e presença de colágeno, edema e proliferação vascular para a dose de 6 mg/kg do corticoide. CONCLUSÃO: Os resultados obtidos demonstram uma relação entre o uso de metilprednisolona por infiltração no espaço subacromial e a redução da resistência mecânica e modificações histológicas no tendão do MR de ratos.