RESUMEN
PURPOSE: To investigate the effects of Periplaneta americana L. on ulcerative colitis (UC) induced by a combination of chronic stress (CS) and 2,4,6-trinitrobenzene sulfonic acid enema (TNBS) in rats. METHODS: The experiment UC model with CS was established in rats by a combination of chronic restraint stress, excess failure, improper, and TNBS. The body weight, disease activity index (DAI), colonic mucosal injury index (CMDI), histopathological score (HS) and pro-inflammatory mediators were measured. The content of corticotropin-releasing hormone (CRH) in hypothalamus or adrenocorticotropic hormone (ACTH) and corticosteroids (CORT) in plasma were evaluated by enzyme-linked immunosorbent assay. The proportion of T lymphocyte subsets was detected by flow cytometry, and gut microbiota was detected by 16S rDNA amplicon sequencing. RESULTS: Weight loss, DAI, CMDI, HS and proinflammatory mediators were reversed in rats by P. americana L. treatment after UC with CS. Increased epidermal growth factor (EGF) was observed in P. americana L. groups. In addition, P. americana L. could reduce the content of CRH and ACTH and regulate the ratio of CD3+, CD3+CD8+ and CD3+CD4+CD25+/CD4+ in spleen. Comparably, P. americana L. changes composition of gut microbiota. CONCLUSIONS: The ethanol extract of Periplaneta Americana L. improves UC induced by a combination of CS and TNBS in rats.
Asunto(s)
Colitis Ulcerosa , Colitis , Periplaneta , Hormona Adrenocorticotrópica/metabolismo , Hormona Adrenocorticotrópica/farmacología , Hormona Adrenocorticotrópica/uso terapéutico , Animales , Colitis/patología , Colitis Ulcerosa/inducido químicamente , Colitis Ulcerosa/tratamiento farmacológico , Colon/patología , Modelos Animales de Enfermedad , Enema , Etanol/farmacología , Extractos Vegetales/farmacología , Extractos Vegetales/uso terapéutico , Ratas , Ácido Trinitrobencenosulfónico/metabolismoRESUMEN
OBJETIVO: Determinar los riesgos y beneficios del uso de vigabatrina comparada con hormona adrenocorticotrópica (ACTH) para el tratamiento de espasmos infantiles. MÉTODO: Se realizó una búsqueda en Epistemonikos. Se extrajeron datos desde las revisiones identificadas. Se realizó un metaanálisis a partir de estudios primarios y se utilizó el método GRADE para la presentación de resultados. RESULTADOS: Se identificaron nueve revisiones sistemáticas. Se observó que el uso de vigabatrina en comparación con ACTH disminuye la resolución de espasmos (RR 0,8, IC 95% 0,65 - 0,98) y podría disminuir la resolución de hipsarritmia (RR 0,71, IC 95% 0,48 - 1,05). No fue posible determinar si el uso de vigabatrina disminuye el riesgo de desarrollar efectos adversos (RR 0,75, IC 95% 0,23 - 2,45) por certeza de evidencia muy baja. CONCLUSIONES: La evidencia parece inclinarse a favor del uso de ACTH. Sin embargo debe considerarse la necesidad de nuevas investigaciones para esclarecer su seguridad.
OBJECTIVE: To determine the risks and benefits of the use of vigabatrin compared to ACTH for the treatment of infantile spasms. METHOD: A search in Epistemonikos was performed. Data were extracted from the identified reviews. A meta-analysis was performed from primary studies and the GRADE method was used to present the results. RESULTS: Nine systematic reviews were identified. Vigabatrin use compared to ACTH was found to decrease resolution of spasms (RR 0.8, 95% CI 0.65 - 0.98) and might decrease resolution of hypsarrhythmia (RR 0.71, 95% CI 0 .48 - 1.05). It was not possible to determine whether the use of vigabatrin reduces the risk of developing adverse effects (RR 0.75, 95% CI 0.23 - 2.45) due to very low certainty of evidence. CONCLUSIONS: The evidence seems to lean in favor of the use of ACTH. However, the need for new research should be considered to clarify its safety.
Asunto(s)
Humanos , Espasmos Infantiles/tratamiento farmacológico , Hormona Adrenocorticotrópica/uso terapéutico , Vigabatrin/uso terapéutico , Anticonvulsivantes/uso terapéutico , Enfoque GRADERESUMEN
Canine pituitary-dependent hypercortisolism (PDH) management with trilostane usually demands lifelong therapy. The greater the dose needed, the greater the risk of side effects. Selegiline therapy has been previously described but not commonly used for PDH treatment. The present work aimed to assess the efficacy of selegiline and trilostane combined therapy for canine PDH treatment. Fifteen client-owned dogs diagnosed with spontaneous PDH were enrolled. The patients were treated with trilostane (Tri group, n = 8, initial dose of 0.5 mg/kg, PO, q12h), or with trilostane and selegiline (Tri + Sel group, n = 7, initial trilostane dose of 0.5 mg/kg, PO, q12h and selegiline 1 mg/kg, PO, q24h). Dogs underwent clinical examination, serum biochemical analysis, urinalysis, abdominal ultrasound, and eACTH and post-ACTH cortisol measurements on treatment days zero (D0), 30 (D30), 90 (D90), and 180 (D180). There was a lack of adverse effects due to the combined therapy. Both groups showed a similar clinical response and lower post-ACTH cortisol levels at the study's end. There was no significant difference in trilostane dosage at D180 between groups. There was no documented increase in either right or left adrenal gland thickness in the Tri + Sel group in contrast with patients in the Tri group. However, there was no statistical difference between the groups regarding eACTH at D0 and D180. Patients in the Tri + Sel group achieved better serum triglycerides control at the end of the study. The association of selegiline with trilostane might be a feasible therapy for canine PDH; however, its eventual advantages need larger studies.
Asunto(s)
Síndrome de Cushing , Enfermedades de los Perros , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT) , Hormona Adrenocorticotrópica/uso terapéutico , Animales , Síndrome de Cushing/veterinaria , Dihidrotestosterona/análogos & derivados , Enfermedades de los Perros/tratamiento farmacológico , Perros , Hidrocortisona , Proyectos Piloto , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/tratamiento farmacológico , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/veterinaria , Selegilina/uso terapéuticoRESUMEN
OBJECTIVES: To characterize and quantify diagnostic and treatment delay among children with infantile spasms, and to estimate the developmental impact of this delay. STUDY DESIGN: In this cohort study, we surveyed the parents of 100 patients with infantile spasms about their experiences with diagnosis and treatment, and ascertained medical and sociodemographic factors potentially related to care of these infants. We specifically determined the latency to first visit an "effective provider," defined as a provider who identified infantile spasms, and prescribed an appropriate first-line treatment, namely adrenocorticotropic hormone, corticosteroids, or vigabatrin. Time to the first visit to an effective provider was evaluated using Cox proportional hazards regression. RESULTS: The median time from the onset of infantile spasms to first visit with an effective provider was 24.5 days. Only 29% of patients were evaluated by an effective provider within 1 week of infantile spasms onset. The time to first effective provider visit was associated with parental language preference, but with no other sociodemographic characteristics. Parents' suspicions that "something is wrong" were often discounted by healthcare providers, and survey respondents frequently reported that pediatricians and neurologists were unfamiliar with infantile spasms. CONCLUSION: This study demonstrates that substantial delay (ie, >1 week) in appropriate care is common, and suggests that the poor awareness of infantile spasms among healthcare providers is at least partly responsible for preventable and potentially significant delays in treatment.
Asunto(s)
Diagnóstico Tardío/estadística & datos numéricos , Espasmos Infantiles/diagnóstico , Corticoesteroides/uso terapéutico , Hormona Adrenocorticotrópica/uso terapéutico , Anticonvulsivantes/uso terapéutico , Competencia Clínica , Electroencefalografía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Los Angeles , Masculino , Neurología , Padres , Pediatría , Relaciones Profesional-Familia , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Espasmos Infantiles/tratamiento farmacológico , Centros de Atención Terciaria , Vigabatrin/uso terapéuticoRESUMEN
OBJECTIVE: Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of hypsarrhythmia. Secondarily, it assesses whether response to treatment differs by etiology or developmental status. METHODS: The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of infantile spasms. Children were considered responders if there was clinical remission and resolution of hypsarrhythmia that was sustained at 3 months after first treatment initiation. Standard treatments of adrenocorticotropic hormone (ACTH), oral corticosteroids, and vigabatrin were considered individually, and all other nonstandard therapies were analyzed collectively. Developmental status and etiology were assessed. We compared response rates by treatment group using chi-square tests and multivariate logistic regression models. RESULTS: Two hundred thirty infants were enrolled from 22 centers. Overall, 46% of children receiving standard therapy responded, compared to only 9% who responded to nonstandard therapy (p < 0.001). Fifty-five percent of infants receiving ACTH as initial treatment responded, compared to 39% for oral corticosteroids, 36% for vigabatrin, and 9% for other (p < 0.001). Neither etiology nor development significantly modified the response pattern by treatment group. INTERPRETATION: Response rate varies by treatment choice. Standard therapies should be considered as initial treatment for infantile spasms, including those with impaired development or known structural or genetic/metabolic etiology. ACTH appeared to be more effective than other standard therapies.
Asunto(s)
Corticoesteroides/administración & dosificación , Hormona Adrenocorticotrópica/uso terapéutico , Anticonvulsivantes/administración & dosificación , Espasmos Infantiles/tratamiento farmacológico , Espasmos Infantiles/epidemiología , Vigabatrin/uso terapéutico , Administración Oral , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Masculino , Prevalencia , Estudios Prospectivos , Factores de Riesgo , Espasmos Infantiles/diagnóstico , Estados Unidos/epidemiologíaRESUMEN
Los glucocorticoides participan en importantes procesos biológicos del organismo. El síndrome de resistencia a los glucocorticoides, o síndrome de Chrousos, es una afección genética causada por mutaciones en el gen del receptor de los glucocorticoides humano. Se caracteriza por la disminución en la sensibilidad tisular al cortisol, con aumento compensatorio en la actividad del eje hipotálamo-hipófisis-adrenal, lo que provoca una elevación de los mineralocorticoides, el cortisol y los andrógenos suprarrenales. El cuadro clínico es variable: asintomático, leve, o con manifestaciones más graves, con hipertensión e hiperandrogenismo. Para el diagnóstico, deben estar ausentes las manifestaciones cushingoides, y demostrarse el hipercortisolismo bioquímico, y la resistencia del eje hipotálamo-hipófisis-adrenal a la supresión con dexametasona. El objetivo del tratamiento es frenar la secreción excesiva de la hormona adrenocorticotropa mediante glucocorticoides que no tengan actividad mineralocorticoide, como es el caso de la dexametasona. En el futuro se espera estén disponibles terapias que permitan corregir directamente los defectos genéticos en el gen del receptor de los glucocorticoides humano(AU)
Glucocorticoids are involved in important biological processes of the body. The glucocorticoid resistance syndrome or Chrousos's syndrome is a genetic disease caused by the human glucocorticoid receptor gene mutations. It is characterized by reduced tissue sensitivity to cortisol and compensatory increase of the hypothalamus-hypophysis-adrenal axis activity, which leads to a rise of minrealocorticoids, cortisol and adrenal androgens levels. The clinical picture is variable, going from asymptomatic, mild or severe manifestations to hypertension and hyperandrogenism. For a right diagnosis, there should be no Cushing-like manifestations and biochemical hypercortisolism as well as hypothalamus-hypophysis-adrenal axis resistance to dexamethasone suppression have to be proven. The objective of the treatment is to halt the excessive secretion of the adrenocorticotrope hormone through glucocorticoids that do not present mineralocorticoid action as it happens with dexamethasone. In the near future, corrective therapies are expected to be available in order to directly fix genetic defects in the human glucocorticoid receptor gen(AU)
Asunto(s)
Humanos , Receptores de Glucocorticoides , Hormona Adrenocorticotrópica/uso terapéutico , Dexametasona/uso terapéutico , SíndromeRESUMEN
Los glucocorticoides participan en importantes procesos biológicos del organismo. El síndrome de resistencia a los glucocorticoides, o síndrome de Chrousos, es una afección genética causada por mutaciones en el gen del receptor de los glucocorticoides humano. Se caracteriza por la disminución en la sensibilidad tisular al cortisol, con aumento compensatorio en la actividad del eje hipotálamo-hipófisis-adrenal, lo que provoca una elevación de los mineralocorticoides, el cortisol y los andrógenos suprarrenales. El cuadro clínico es variable: asintomático, leve, o con manifestaciones más graves, con hipertensión e hiperandrogenismo. Para el diagnóstico, deben estar ausentes las manifestaciones cushingoides, y demostrarse el hipercortisolismo bioquímico, y la resistencia del eje hipotálamo-hipófisis-adrenal a la supresión con dexametasona. El objetivo del tratamiento es frenar la secreción excesiva de la hormona adrenocorticotropa mediante glucocorticoides que no tengan actividad mineralocorticoide, como es el caso de la dexametasona. En el futuro se espera estén disponibles terapias que permitan corregir directamente los defectos genéticos en el gen del receptor de los glucocorticoides humano(AU)
Glucocorticoids are involved in important biological processes of the body. The glucocorticoid resistance syndrome or Chrousos's syndrome is a genetic disease caused by the human glucocorticoid receptor gene mutations. It is characterized by reduced tissue sensitivity to cortisol and compensatory increase of the hypothalamus-hypophysis-adrenal axis activity, which leads to a rise of minrealocorticoids, cortisol and adrenal androgens levels. The clinical picture is variable, going from asymptomatic, mild or severe manifestations to hypertension and hyperandrogenism. For a right diagnosis, there should be no Cushing-like manifestations and biochemical hypercortisolism as well as hypothalamus-hypophysis-adrenal axis resistance to dexamethasone suppression have to be proven. The objective of the treatment is to halt the excessive secretion of the adrenocorticotrope hormone through glucocorticoids that do not present mineralocorticoid action as it happens with dexamethasone. In the near future, corrective therapies are expected to be available in order to directly fix genetic defects in the human glucocorticoid receptor gen(AU)
Asunto(s)
Humanos , Dexametasona/uso terapéutico , Hormona Adrenocorticotrópica/uso terapéutico , Receptores de GlucocorticoidesRESUMEN
We describe two familial and three nonfamilial cases from Argentina, examined between February 1, 1990-July 31, 2008, who met the diagnostic criteria of progressive encephalopathy, peripheral edema, hypsarrhythmia, and optic atrophy syndrome. All five children were products of normal gestation, although one was premature. Birth was uneventful in all patients. Two patients were twin brothers. During their first neurologic examination, between ages 2-6 months, patients presented with facial dysmorphia, poor visual contact, and generalized hypotonia with poor head control. Microcephaly and swelling of the dorsum of the hands and feet were evident. Hypsarrhythmia was observed in all patients (associated with epileptic spasms in four). Optic atrophy was evident in four cases. Magnetic resonance imaging indicated progressive cerebellum and brainstem atrophy in all cases. Toxoplasmosis, others, rubella, cytomegalovirus, herpes (TORCH), neurometabolic investigations, and karyotype studies produced normal results in all patients. Progressive encephalopathy, peripheral edema, hypsarrhythmia, and optic atrophy syndrome should be considered in infants with neonatal hypotonia, early onset of seizures (especially epileptic spasms), hypsarrhythmia, early loss of visual fixation, profound psychomotor retardation, typical dysmorphy, and progressive cerebellar and brainstem atrophy without a clear etiology. Autosomal recessive inheritance is suspected. Early diagnosis is important for adequate genetic counseling.
Asunto(s)
Edema Encefálico , Enfermedades Neurodegenerativas , Atrofia Óptica , Espasmos Infantiles , Hormona Adrenocorticotrópica/uso terapéutico , Argentina , Edema Encefálico/diagnóstico , Edema Encefálico/fisiopatología , Diagnóstico por Imagen/métodos , Electroencefalografía , Epilepsia/fisiopatología , Femenino , Hormonas/uso terapéutico , Humanos , Lactante , Masculino , Hipotonía Muscular/etiología , Enfermedades Neurodegenerativas/diagnóstico , Enfermedades Neurodegenerativas/fisiopatología , Atrofia Óptica/diagnóstico , Atrofia Óptica/fisiopatología , Espasmos Infantiles/diagnóstico , Espasmos Infantiles/fisiopatologíaRESUMEN
BACKGROUND: High cortisol levels are frequent in patients with severe infections. However, the predictive value of total cortisol and of the presence of critical illness-related corticosteroid insufficiency (CIRCI) in severe community-acquired pneumonia (CAP) remains to be thoroughly evaluated. The aim of this study was to investigate the predictive value of adrenal response in patients with severe CAP admitted to the ICU. METHODS: Baseline and postcorticotropin cortisol levels C-reactive protein (CRP), d-dimer, clinical variables, sequential organ failure assessment (SOFA), APACHE (acute physiology and chronic health evaluation) II, and CURB-65 (confusion, urea nitrogen, respiratory rate, BP, age > or = 65 years) scores were measured in the first 24 h. Results are shown as median (interquartile range [IQR]). The major outcome measure was hospital mortality. RESULTS: Seventy-two patients with severe CAP admitted to the ICU were evaluated. Baseline cortisol levels were 18.1 microg/dL (IQR, 14.4 to 26.7 microg/dL), and the difference between baseline and postcorticotropin cortisol after 250 microg of corticotropin was 19 microg/dL (IQR, 12.8 to 27 microg/dL). Baseline cortisol levels presented positive correlations with scores of disease severity, including CURB-65, APACHE II, and SOFA (p < 0.05). Cortisol levels in nonsurvivors were higher than in survivors. CIRCI was diagnosed in 29 patients (40.8%). In univariate analysis, baseline cortisol, CURB-65, and APACHE II were predictors of death. The discriminative ability of baseline cortisol (area under receiver operating characteristic curve, 0.77; 95% confidence interval, 0.65 to 0.90; best cutoff for cortisol, 25.7 microg/dL) for in-hospital mortality was better than APACHE II, CURB-65, SOFA, d-dimer, or CRP. CONCLUSIONS: Baseline cortisol levels are better predictors of severity and outcome in severe CAP than postcorticotropin cortisol or routinely measured laboratory parameters or scores as APACHE II, SOFA, and CURB-65.
Asunto(s)
Glándulas Suprarrenales/metabolismo , Hidrocortisona/sangre , Neumonía Bacteriana/sangre , Hormona Adrenocorticotrópica/administración & dosificación , Hormona Adrenocorticotrópica/uso terapéutico , Adulto , Anciano , Anciano de 80 o más Años , Brasil/epidemiología , Infecciones Comunitarias Adquiridas/sangre , Infecciones Comunitarias Adquiridas/tratamiento farmacológico , Infecciones Comunitarias Adquiridas/mortalidad , Relación Dosis-Respuesta a Droga , Femenino , Estudios de Seguimiento , Hormonas/administración & dosificación , Hormonas/uso terapéutico , Mortalidad Hospitalaria , Humanos , Masculino , Persona de Mediana Edad , Neumonía Bacteriana/tratamiento farmacológico , Neumonía Bacteriana/mortalidad , Pronóstico , Curva ROC , Unidades de Cuidados Respiratorios , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
Se realizó una cohorte en niños con Síndrome de West (SW) atendidos en Neuropediatría del Hospital Belén de Trujillo, entre enero de 1994 a Julio del 2007. Los objetivos fueron: determinar las características clínicas, etiológicas, evolución, y comparar la eficacia de la ACTH con otras drogas antiepilépticas (DAE) en el tratamiento del SW. La muestra fue de 30 niños: 1. Espasmos de flexión, retraso psicomotor y electroencefalograma con espasmo-supresión o hipsarrítmia caracterizan al Síndrome de West. 2. La etiologia sintomática (80%) predominó sobre idiopáticas y criptogénicas. 3.La curación de los espasmos epilépticos en el SW ocurrió en 14 niños (46.6%). 4.Todos los niños con SW recibieron otras drogas antiepilépticas y curaron 6/30 (20%), 14 recibieron ACTH y curaron 8/14 (57.1%) (p<0.05). 5.El Síndrome de Lennox Gastaut ocurrió en 8 niños (26.6%), todos, excepto uno tuvieron previamente SW; solamente 2/30 niños (6.6%) alcanzaron la normalidad cognitiva. En conclusión el SW se caracteriza por: espasmos de flexión, retraso psicomotor y EEG con espasmosupresión e hipsarritmia, predomina la etiologia sintomática, curación de 46.6%, la ACTH es la DAE más eficaz, baja posibilidad de alcanzar la normalidad cognitiva y evolucionan al síndrome de Lennox Gastaut.
It realized a cohort study in children with West syndrome that attended in Neuropediatrícs at Belen Hospital of Trujillo, between January 1994 and December 2006. Objectives were: establish the clínic characteristic, etiology, evolution, to compare efficacy of ACTH with others anti epilepsy drugs at West syndrome treatment. The sample was 30 children.The results are:1. The flexion spasms, psychomotor retard and electroencephalogram with suppression spasms or hypsarrhythmia characterize the West syndrome. 2. The symptomatic etiology was (80%) predominated upon the idiopathic and cryptogenic. 3. The epilepsy spasms remíssion (cure) in the West syndrome was 46.6%. 4. All children with West syndrome received antiepilepsy drugs, were cured 6/30 (20%), 14 children receíved ACTH and were cured 8/14 (57.1%) (p<0.05). 5. The Lennox Gastaut syndrome occurred in 26.6%. Everybody, except one had previously West syndrome, only two children (6.6%) recuperated their normality.The following ends that the West syndrome characterized for flexion spasms, psychomotor retard and EEG with suppression spasms and hypsarrhythmia, the symptomatic etiology ís predominated, 50% approach to cure, the ACTH is the most efficacious DAE, evolution to the Lennox Gastaut syndrome and they had low possibility to recuperate the cognitive normality.
Asunto(s)
Masculino , Femenino , Humanos , Niño , Anticonvulsivantes/uso terapéutico , Epilepsia/terapia , Espasmos Infantiles/etiología , Espasmos Infantiles/terapia , Hormona Adrenocorticotrópica/uso terapéutico , Estudios de CohortesRESUMEN
INTRODUCTION: Landau-Kleffner syndrome is characterised by acquired aphasia and encephalographic alterations that may or may not be accompanied by epileptic seizures. AIM. To analyse the clinical and encephalographic features and response to treatment of 10 patients with Landau-Kleffner syndrome. PATIENTS AND METHODS: We reviewed the patient records, encephalograms and treatment administered to patients catalogued as having Landau-Kleffner syndrome. RESULTS: The mean age of the patients was 44 months. Of these cases, 60% presented epilepsy when the diagnosis was established and 70% were found to have epileptic status during slow-wave sleep in the encephalographic study. Results showed that 40% corresponded to variants of Landau-Kleffner syndrome. No cause of the disease could be established in any of the patients. In the neuroimaging study, only one patient displayed abnormalities in the magnetic resonance imaging of the brain. All the patients received adrenocorticotropic hormone (ACTH)-based treatment, at a dose of 1 IU/kg/day for one month, administered together with antiepileptic drugs such as valproic acid and clobazam. Convulsive seizures and epileptic status during slow-wave sleep disappeared in all the patients. In the patients without epileptic status, epileptic activity became less frequent, although it did not completely disappear. Aphasia improved considerably, which meant that all the patients were able to enroll in normal schools. CONCLUSIONS: We believe that early diagnosis, together with suitable and timely management of aphasic patients with encephalographic alterations that allow ACTH to be used at low doses, make it possible to offer an early education so as to provide maximum recovery from the disease.
Asunto(s)
Síndrome de Landau-Kleffner , Convulsiones , Hormona Adrenocorticotrópica/uso terapéutico , Anticonvulsivantes/uso terapéutico , Afasia/diagnóstico , Afasia/tratamiento farmacológico , Afasia/fisiopatología , Preescolar , Electroencefalografía , Humanos , Lactante , Síndrome de Landau-Kleffner/complicaciones , Síndrome de Landau-Kleffner/diagnóstico , Síndrome de Landau-Kleffner/tratamiento farmacológico , Síndrome de Landau-Kleffner/fisiopatología , Estudios Retrospectivos , Convulsiones/tratamiento farmacológico , Convulsiones/etiología , Convulsiones/fisiopatologíaRESUMEN
OBJECTIVES: To evaluate the outcome of West syndrome and to elucidate the differences in the outcome related to the timing of brain injury. STUDY DESIGN: Medical records of 60 patients who were followed regularly for more than seven years were reviewed. The following clinical features were assessed: onset, seizure evolution, electroencephalography and intelligence. Those variables were compared among five groups: cryptogenic, prenatal, preterm, term, and postnatal groups. RESULTS: The onset ages of the postnatal group were later than those of the others (P<.05). The relapse after adrenocorticotropic hormone therapy of the preterm group was the earliest among the groups (P<.05). Regarding encelphalography, the ratio of patients with focal discharges was higher in the postnatal group than in the prenatal group (P<.05). The ratios of patients in whom focal epilepsy developed were higher in the term and postnatal group than in the cryptogenic and prenatal group (P<.05). The term group showed similar characteristics to those of the postnatal group. Seven of the 60 had normal intelligence, including three girls with tuberous sclerosis. CONCLUSION: The diverse outcomes of West syndrome depending on etiology seemed to be related to the timing of brain injury and brain development.
Asunto(s)
Espasmos Infantiles/fisiopatología , Adolescente , Hormona Adrenocorticotrópica/uso terapéutico , Edad de Inicio , Encéfalo/embriología , Niño , Electroencefalografía , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Inteligencia , Masculino , Registros Médicos , Estudios Retrospectivos , Espasmos Infantiles/congénito , Espasmos Infantiles/tratamiento farmacológico , Espasmos Infantiles/embriología , Espasmos Infantiles/etiologíaRESUMEN
Two cases are described of children who at first developed normally, but before the age of three developed autistic symptoms following the reactivation of a chronic oto-rhinolaryngologic infection. The clinical and laboratory data of the cases support the aetiological hypothesis of an autoimmune process. Adrenocorticotrophic hormone (ACTH), prescribed in the first months of the disease, cured one case. The other patient, who was two years old when autistic symptoms appeared and was treated only six years later, showed a partial but definitive improvement with the immunosuppressive treatment. This report proposes that reactivation of a chronic bacterial infection be included among the aetiologies of Late Onset Autism, and demonstrates that, when the aetiological hypothesis of an autoimmune process based on clinical and laboratory data is considered, an immunosuppressive treatment, particularly with ACTH, can be very effective and also safe.
Asunto(s)
Hormona Adrenocorticotrópica/uso terapéutico , Antiinflamatorios/uso terapéutico , Autoanticuerpos/inmunología , Inmunosupresores/uso terapéutico , Prednisolona/uso terapéutico , Adolescente , Factores de Edad , Trastorno Autístico/tratamiento farmacológico , Trastorno Autístico/inmunología , Trastorno Autístico/microbiología , Infecciones Bacterianas/complicaciones , Encéfalo/inmunología , Femenino , Humanos , MasculinoRESUMEN
A 39-year-old man presented with 80% body surface area burned following a thermal accident. The patient showed hemodynamic instability and low response to intravenous fluids or vasopressors for 20 days in the intensive care unit (ICU). The adrenocorticotropic hormone (ACTH) test was consistent with adrenal insufficiency. After a 3-day steroid treatment, the patient's blood pressure was normal without the administration of any vasopressor.
Asunto(s)
Insuficiencia Suprarrenal/etiología , Hormona Adrenocorticotrópica/uso terapéutico , Quemaduras/complicaciones , Hemodinámica/efectos de los fármacos , Hidrocortisona/uso terapéutico , Pruebas de Función de la Corteza Suprarrenal , Adulto , Quemaduras/terapia , Fluidoterapia , Humanos , Masculino , Factores de Tiempo , Vasoconstrictores/uso terapéutico , Cicatrización de HeridasAsunto(s)
Humanos , Lactante , Femenino , Espasmos Infantiles/complicaciones , Cuerpo Calloso/anomalías , Anomalías del Ojo/complicaciones , Espasmos Infantiles/diagnóstico , Espasmos Infantiles/etiología , Coriorretinitis/complicaciones , Coriorretinitis/etiología , Hormona Adrenocorticotrópica/uso terapéutico , Fondo de Ojo , Discapacidad Intelectual/complicaciones , Discapacidad Intelectual/etiologíaRESUMEN
Forty-one cases of Cushing's Disease affecting both equine and canine patients were treated with an identical mixture of two homeopathically prepared remedies (ACTH 30c and Quercus robur 30c), and the clinical improvements seen in the cases assessed. Homeopathy has been described as a medicine that can only be prescribed on the basis of... (AU)
Asunto(s)
Animales , Perros , Bovinos , Síndrome de Cushing/terapia , Hormona Adrenocorticotrópica/uso terapéutico , HomeopatíaRESUMEN
In a retrospective study we assessed the outcome of the criptogenic and symptomatic forms of West syndrome and evaluated the efficacy of adrenocorticotropic hormone, vigabatrin, prednisone, valproate and nitrazepam in the spasms control. Seventy patients were follwed up by 2 years. Twelve (17%) were criptogenics and 58 (83%) symptomatics. In criptogenic group significantly more patients were in regular school classes and with normal motor development, better control of seizure, less tendency to evoluate to Lennox Gastaut syndrome and 83. 3% had control of spasms (72.4% of patients from symptomatic group had control of spasms). Adrenocorticotropic hormone and vigabatrin were the most efective drugs, with 68.75% and 60% of spasms control, respectivelly, when used as first line of therapy and 75% and 50%, respectivelly, as second line of therapy.