Encefalitis autoinmune posherpética. Caso clínico pediátrico / Autoimmune post-herpes simplex encephalitis. A pediatric clinical case report

La encefalitis por virus herpes simple (VHS) es una causa frecuente de encefalitis grave y potencialmente fatal. La encefalitis autoinmune posherpética (EAPH) afecta a un porcentaje de los pacientes que han presentado encefalitis herpética (EH) y se caracteriza por la aparición de nuevos síntomas neurológico/psiquiátricos, y/o por el empeoramiento de los déficits adquiridos durante la infección viral dentro de un lapso temporal predecible. Se produce por un mecanismo no relacionado con el VHS, sino por fenómenos autoinmunes, y es susceptible de tratamiento con inmunomoduladores. Se presenta el caso de un varón de 5 años de edad con EAPH que requirió tratamiento inmunomodulador, de primera y segunda línea, con buena evolución y remisión de los síntomas.

Herpes simplex virus (HSV) encephalitis is a common cause of severe and potentially fatal encephalitis. Autoimmune post-herpes simplex encephalitis (AIPHSE) affects a percentage of patients who developed herpes simplex encephalitis (HSE) and is characterized by the onset of new neurological/psychiatric symptoms and/or worsening of deficits acquired during the herpes infection within a predictable time frame. It is caused by a mechanism not related to HSV, but by autoimmune conditions, and is susceptible to treatment with immunomodulators. Here we describe the case of a 5-year-old boy with AIPHSE who required first- and second-line immunomodulatory treatment, with an adequate course and remission of symptoms.

Papel inmunomodulador de la vitamina D y los ácidos grasos poliinsaturados omega-3 en trastornos autoinmunes: Revisión de la Literatura / Immunomodulatory role of vitamin D and omega-3 polyunsaturated fatty acids in autoinmune disorders: Literature Review

Los trastornos autoinmunes representan una familia de al menos 80 condiciones diferentes que surgen de una respuesta aberrante del sistema inmunológico resultando finalmente en la destrucción de tejidos y órganos específicos del cuerpo. Es importante destacar que durante las últimas tres décadas los estudios epidemiológicos han proporcionado evidencia de un aumento constante en la incidencia y prevalencia de trastornos autoinmunes. En los últimos años, varios estudios han demostrado que la vitamina D y los ácidos grasos poliinsaturados (AGPs) omega-3 ejercen propiedades inmunomoduladoras y antiinflamatorias sinérgicas que pueden aprovecharse positivamente para la prevención y el tratamiento de trastornos autoinmunes. En este sentido, el reciente ensayo clínico denominado VITAL (ensayo de vitamina D y omega 3); un estudio a gran escala, aleatorizado, doble ciego, controlado con placebo encontró que la suplementación conjunta de vitamina D y AGPs omega-3 (VIDOM) puede reducir la incidencia de enfermedades autoinmunes. En esta revisión de la literatura, resumimos los mecanismos moleculares detrás de las propiedades inmunomoduladoras y antiinflamatorias de la vitamina D y los AGPs omega-3, así como la posible interacción bidireccional entre el metabolismo de la vitamina D y el metabolismo de los AGPs omega-3 que justifica la co- suplementación VIDOM en trastornos autoinmunes(AU)

Autoimmune disorders represent a family of at least 80 different conditions that arise from an aberrant immune system response, which ultimately results in the destruction of specific body tissues and organs. It is important to highlight that during the last three decades epidemiological studies have provided evidence of a steady increase in the incidence and prevalence of autoimmune disorders. In recent years, several studies have shown that vitamin D and omega-3 polyunsaturated fatty acids (PUFAs) exert synergistic immunomodulatory and anti-inflammatory properties that can be positively harnessed for the prevention and treatment of autoimmune disorders. In this sense, the recent clinical trial called VITAL (Vitamin D and Omega 3 trial) - a large, randomized, double-blind, placebo- controlled study - found that co-supplementation of vitamin D and omega-3 PUFAs (VIDOM) can reduce the incidence of autoimmune diseases. In this literature review, we summarize the molecular mechanisms behind the immunomodulatory and anti-inflammatory properties of vitamin D and omega-3 PUFAs, as well as the possible bidirectional interaction between vitamin D metabolism and omega-3 PUFA metabolism that justifies VIDOM co- supplementation in autoimmune disorders(AU)

Fenómeno infrecuente la co-localización de alopecia frontal fibrosante y vitiligo en un varón: informe de un caso / Infrequent phenomenon the co-localization of fibrosing frontal alopecia and vitiligo in a male: case repor

La alopecia frontal fibrosante es una alopecia cicatricial que se caracteriza por la recesión de la línea de implantación frontotemporal que afecta principalmente a mujeres caucásicas en edad posmenopáusica y rara vez a hombres. Actualmente los mecanismos específicos de desarrollo continúan en estudio; sin embargo hay varias hipótesis sobre la asociación de la alopecia frontal fibrosante con otros trastornos autoinmunitarios. Se comunica el caso de un paciente masculino de 58 años con alopecia frontal fibrosante en áreas comprometidas por vitiligo. (AU)

Frontal fibrosing alopecia is a cicatricial alopecic characterized by progressive regression of the frontotemporal hairline. It usually affects postmenopausal caucasian women, and rarely men. Currently the specific mechanisms of development remain unknown, however there are several hypotheses about the association of frontal fibrosing alopecia with other autoimmune disorders. The case of a 58-year-old male patient with frontal fibrosing alopecia in areas affected by vitiligo. (AU)

Insuficiencia ovárica primaria en adolescentes: revisión de la literatura a propósito de un caso clínico / Primary ovarian insufficiency in adolescents: review of the literature regarding a clinical case

El objetivo de este manuscrito es realizar una revisión y actualización de la literatura de la insuficiencia ovárica primaria (IOP) en población adolescente, a partir del diagnóstico, manejo y seguimiento de un caso clínico. La insuficiencia ovárica primaria se define como la menopausia en una mujer antes de los 40 años, acompañada de amenorrea, hipogonadismo hipergonadotrópico e infertilidad. Su prevalencia varía entre 1 a 2%, y en mujeres menores de 20 años su prevalencia es un caso de cada 10,000. Aunque se sabe que muchas afecciones pueden llevar a una IOP, la más común es la causa idiopática. La presentación clínica es diversa, y varios trastornos diferentes pueden también, llevar a esta condición. CASO CLÍNICO: Se presenta el caso de una adolescente de 17 años, previamente sana, con historia de amenorrea secundaria, no embarazada, con examen físico general y ginecológico normal. Se solicita estudio analítico complementario resultando con niveles de hormona folículo estimulante (FHS), estradiol (E2) y hormona antimülleriana (AMH) compatibles con una insuficiencia ovárica como la observada en la posmenopausia. Se inicia terapia hormonal (TH) clásica con estradiol y progesterona, siendo posteriormente reemplazada por anticoncepción hormonal combinada (AHC) oral, coincidente con el inicio de vida sexual, con respuesta favorable y sangrados regulares. La IOP tiene graves consecuencias para la salud incluyendo trastornos psicológicos como angustia, síntomas depresivos o depresión, infertilidad, osteoporosis, trastornos autoinmunes, cardiopatía isquémica, y un mayor riesgo de mortalidad. La enfermedad de Hashimoto es el trastorno autoinmune más frecuente asociado a la IOP. Su tratamiento y diagnóstico deben establecerse de forma precoz para evitar consecuencias a largo plazo. La terapia con estrógenos es la base del tratamiento para eliminar los síntomas de la deficiencia de estrógenos, además de evitar las consecuencias futuras del hipogonadismo no tratado. También el manejo debe incluir los siguientes dominios: fertilidad y anticoncepción, salud ósea, problemas cardiovasculares, función psicosexual, psicológica y neurológica, informando a los familiares y a la paciente sobre la dimensión real de la IOP y la necesidad de tratamiento multidisciplinario en muchos casos. CONCLUSIÓN: El caso presentado, pese a ser infrecuente, permite abordar de manera sistematizada el diagnostico de IOP y evaluar alternativas de manejo plausibles para evitar graves consecuencias en la salud, así como conocer respuesta clínica y de satisfacción de la adolescente.

The objective of this manuscript is to review and update the literature on primary ovarian insufficiency (POI) in an adolescent population, based on the diagnosis, management and follow-up of a clinical case. Primary ovarian insufficiency is defined as menopause in a woman before the age of 40, accompanied by amenorrhea, hypergonadotropic hypogonadism, and infertility. Its prevalence varies between 1 to 2%, and in women under 20 years of age its prevalence is one case in every 10,000. Although it is known that many conditions can lead to POI, the most common is the idiopathic cause. The clinical presentation is diverse, and several different disorders can also lead to this condition. CLINICAL CASE: The case of a 17-year-old adolescent, previously healthy, with a history of secondary amenorrhea, not pregnant, with a normal general physical and gynecological examination is presented. A complementary analytical study is requested, resulting in levels of follicle stimulating hormone (FHS), estradiol (E2) and anti-müllerian hormone (AMH) compatible with ovarian insufficiency such as that observed in postmenopause. Classic hormonal therapy (HT) with estradiol and progesterone was started, later being replaced by combined hormonal contraception (CHC), coinciding with the beginning of sexual life, with a favorable response and regular bleeding. POI has serious health consequences including psychological disorders such as distress, depressive symptoms or depression, infertility, osteoporosis, autoimmune disorders, ischemic heart disease, and an increased risk of mortality. Hashimoto's disease is the most common autoimmune disorder associated with POI. Its treatment and diagnosis must be established early to avoid long-term consequences. Estrogen therapy is the mainstay of treatment to eliminate the symptoms of estrogen deficiency, in addition to avoiding the future consequences of untreated hypogonadism. Management should also include the following domains: fertility and contraception, bone health, cardiovascular problems, psychosexual, psychological and neurological function, informing family members and the patient about the real dimension of POI and the need for multidisciplinary treatment in many cases. CONCLUSION: The case, although infrequent, allows a systematic approach to the diagnosis of POI and evaluate plausible management alternatives to avoid serious health consequences, as well as to know the clinical response and satisfaction of the adolescent.

Advance in autoimmune diseases related to Turner syndrome / 国际儿科学杂志

Turner syndrome(TS)results from a sex-chromosomal anomaly characterized by presence of one normal X chromosome and the loss of the second X-chromosome in phenotypic females.The typical clinical manifestations of Turner syndrome are short stature, hypogonadal dysplasia, and special physical characteristics(such as neck web, shield chest, elbow valgus, etc.). And it is related to the risk of autoimmune diseases, such as autoimmune thyroiditis, celiac disease, type 1 diabetes, inflammatory bowel disease, alopecia areata or vitiligo.This review will address the autoimmune disorders associated with TS, their pathophysiologic mechanisms and clinical characteristics.

Recent advances in myeloid-derived suppressor cell biology / 医学前沿

In recent years, studying the role of myeloid-derived suppressor cells (MDSCs) in many pathological inflammatory conditions has become a very active research area. Although the role of MDSCs in cancer is relatively well established, their role in non-cancerous pathological conditions remains in its infancy resulting in much confusion. Our objectives in this review are to address some recent advances in MDSC research in order to minimize such confusion and to provide an insight into their function in the context of other diseases. The following topics will be specifically focused upon: (1) definition and characterization of MDSCs; (2) whether all MDSC populations consist of immature cells; (3) technical issues in MDSC isolation, estimation and characterization; (4) the origin of MDSCs and their anatomical distribution in health and disease; (5) mediators of MDSC expansion and accumulation; (6) factors that determine the expansion of one MDSC population over the other; (7) the Yin and Yang roles of MDSCs. Moreover, the functions of MDSCs will be addressed throughout the text.

Evaluation of plasma soluble CD137 level in relapsing-remitting multiple sclerosis patients in comparison with healthy controls in Isfahan Province, Iran

@#Objective: Multiple sclerosis (MS) is a chronic neuroinflammatory disease, characterizes by demyelination in the central nervous system (CNS). Co-stimulatory molecules such as CD137 (4-1 BB) play a major role in the activation of lymphocytes in CNS. The exact immunopathogenesis of MS is unknown. Hence, detection of specific biomarkers in the process of MS disease can lead to new therapeutic approaches. This study aimed to compare plasma sCD137 levels in relapsing-remitting multiple sclerosis (RRMS) patients with healthy controls in Isfahan province. Methods: Plasma sCD137 level was measured by enzyme-linked immune sorbent assays (ELISA) in 36 RRMS patients as well as 52 (age and sex-matched) healthy controls and the results were compared. Results: The plasma sCD137 level in studied RRMS patients was significantly higher in the patient group compared to the healthy controls (P- value=0.027). In addition, there was no significant association between age, sex, job and education level, with plasma sCD137 level in both the control and the case groups (P value>0.05). There was no correlation between mean of sCD137 and EDSS score, age of onset, duration of disease as well as serum 25 (OH) D concentrations of the patients. Conclusion: High plasma sCD137 level was detected in RRMS patients when compared with the controls, which may indicate the possible role of this biomarker in the immunopathogenesis of MS. Since CD137 can affect T lymphocytes activation and apoptosis, further studies are needed to elucidate its exact role in the pathogenesis of MS.

Rheumatology Panel in Pediatric Practice

Common rheumatological disorders encountered in pediatric practice are juvenile idiopathic arthritis, systemic juvenile idiopathicarthritis, Kawasaki disease, Henoch-Schonlein purpura, systemic lupus erythematosus, chronic uveitis and juvenile dermatomyositis.Diagnosis of these disorders requires a critical appraisal of the clinical history, physical examination and relevant investigations.Laboratory tests are helpful for screening purposes as also for confirmation of diagnosis and monitoring of disease activity. These testsshould, however, only be ordered after due deliberation and in the context of clinical findings in a given patient.

Características clínicas de pacientes con déficit selectivo de IgA en el hospital infantil municipal de Córdoba, periodo 2010-2013 / Clinical features of patients with selective Iga deficiency In municipal hospital children of Córdoba, period 2010-2013

Introducción: el déficit selectivo de IgA (DsIgA) es la inmunodeficiencia primaria más frecuente, caracterizada por nivelesséricos de inmunoglobulina A (IgA) menores a 7 mg/dl en pacientes mayores a 4 años con niveles normales de otros isotipos de inmunoglobulinas. Aunque la mayoría de los individuos sona sintomáticos, cerca de un tercio padecen infecciones recurrentes, alergia o autoinmunidad. Objetivo: Principal: determinar características clínicas asociadas al DsIgA. Secundario: analizar los valores de inmunoglobulina G (IgG) e inmunoglobulina M (IgM) de los pacientes.Materiales y métodos: Se revisaron en forma retrospectiva registros de laboratorio e historias clínicas de 24 pacientes que cumplieron con los criterios diagnósticos de DsIgA. Resultados: Se observó que 17 pacientes (71%) presentaban fenó-menos alérgicos, 13 pacientes (54%) enfermedades autoinmunes y 7 pacientes (29%) infecciones recurrentes al momento de diagnóstico del DsIgA. Además se pudo ver un aumento de IgG e IgMen un 38% y 46% de los pacientes, respectivamente. Conclusión: Observamos que la principal manifestación clínica fue la alergia, y que un grupo de pacientes tenía elevadas sus inmunoglobulinas séricas.

Introduction: selective IgA deficiency (IgAD) is considered as the most common primary immunodeficiency, is defined as a serum IgA of less than 7mg/dl, with normal serum IgG and IgM levels in patients older than 4 years with other wise normal immune system. Although the majority of affected individuals are asymptomatic, a third of patients are symptomatic and suffer from recurrent infections, allergies and autoimmune diseases. Aim: To investigate the clinical characteristics of children with IgAD and analyse the levels of IgG and IgM of the patients. Materials and methods: The clinical and laboratory information from medical records of 24 children with IgAD were studied retrospectively. Results: 17 (71%) patients had allergic disorders, 13 (54%) patients had autoimmune disorders and 7 (29%) patients had infectious diseases at the moment of the diagnosis of IgAD. Serum IgG and IgM levels were increased in 38 % and 46 % of patients, respectively. Conclusions: we found that allergic disorders were the most common clinical manifestation and group of patients had increased levels of serum immunoglobulins.

Hepatitis C and cutaneous alterations / Hepatite C e alterações cutâneas

While most of those infected with hepatitis C virus (HCV) are asymptomatic or only develop liver manifestations, a significant percentage evolves with autoimmune and lymphoproliferative disorders, resulting in a clinical condition called HCV syndrome. This work involving case studies of six patients with hepatitis C and varied skin manifestation aimed to report skin lesions occurring with HCV infection and its treatment. Skin manifestations in hepatitis C have been based on epidemiological studies. This justifies the need for studies that correlate HCV infection and its treatment with skin manifestations.

A maioria dos infectados pelo vírus da hepatite C (VHC) é assintomática ou apresenta somente manifestações hepáticas, porém uma significativa porcentagem evolui com desordens autoimunes e linfoproliferativas resultando na síndrome da hepatite C. Este trabalho envolve seis estudos de casos de pacientes com hepatite C e várias manifestações cutâneas, visando relacionar lesões dermatológicas ao VHC e ao seu tratamento. Manifestações dermatológicas na hepatite C têm sido baseadas em estudos epidemiológicos. Isto justifica a necessidade de estudos que correlacionem infecção pelo VHC e seu tratamento com as manifestações dermatológicas.

Manifestaciones Extrahepaticas de la Hepatitis C. Reporte de un Caso / Extrahepatic Manifestations of Hepatitis C. Report one Case

RESUMEN Reportamos 1 caso de hepatitis C en una paciente adulta sin antecedentes de riesgos previos. Se describe una manifestación extrahepática, afectación de sistema nervioso central por mecanismos autoinmunes, lo cual determina la indicación de tratamiento, aún sin cumplir con los criterios de biopsia hepática y de carga viral.

ABSTRACT We report 1 case of hepatitis C in an adult patient with no history of previous risks. Describes an extrahepatic manifestation, involvement of the central nervous system autoimmune mechanisms, which determines the indication of treatment, even without meeting the criteria for liver biopsy and viral load.

Diabetes e doenças auto-imunes: prevalência de doença celíaca em crianças e adolescentes portadores de diabetes melito tipo 1 / Diabetes and autoimmune diseases: prevalence of celiac disease in children and adolescents with type 1 diabetes

OBJETIVO: Determinar a prevalência de doença celíaca em crianças e adolescentes portadores de diabetes melito tipo1 (DM1) no Instituto Estadual de Diabetes e Endocrinologia Luiz Capriglione (IEDE). MÉTODOS: Foram analisadas amostras de sangue de 120 crianças e adolescentes portadores de DM1 do Ambulatório de Diabetes do IEDE para a pesquisa do anticorpo antitransglutaminase tecidual humana IgA e dosagem da IgA sérica. Aqueles com sorologia positiva foram encaminhados para endoscopia digestiva alta com biópsia de intestino delgado para a confirmação da doença celíaca. RESULTADOS: O anticorpo foi positivo em três dos 120 pacientes analisados. Todos os positivos apresentaram biópsia de intestino delgado confirmatória para doença celíaca, gerando prevalência desta doença no grupo estudado de 2,5 por cento. CONCLUSÃO: A prevalência de doença celíaca encontra-se aumentada entre crianças e adolescentes com DM1 acompanhadas no IEDE quando comparadas à normalidade. Como a maioria é assintomática, recomenda-se o rastreamento periódico desta doença em todas as crianças com DM1.

OBJECTIVE: Determinate the prevalence of celiac disease in children and adolescents with type 1 diabetes mellitus (DM1) in attendance in Instituto Estadual de Diabetes e Endocrinologia Luiz Capriglione (IEDE). METHODS: Blood samples were analyzed in 120 children and adolescents with DM1 from IEDE Diabetes Clinic for the IgA antitissue-transglutaminase antibody and dosage of the seric IgA. Those with positive serology were guided for upper endoscopy with small-bowel biopsy to confirm the celiac disease. RESULTS: The antibody was positive in 3 of the 120 patients. The small-bowel biopsy was confirmatory in all of the positive patients, leading to a prevalence of celiac disease of 2.5 percent in the studied group. CONCLUSIONS: The prevalence of celiac disease is increased in children and adolescents with DM1 when compared with normality. As most are asymptomatic, it is recommended periodical screening of celiac disease in children with DM1.

The Clinical Significance of Detecting Nonthyroid Specific Antibodies in Patients with Graves' Diseases / 中国慢性病预防与控制

Objective To study the level of immunoglobulin and the prevalence of ANA in patients with Graves' diseases(GD).To explore the correlation between GD and other systemic autoimmune disorders.Methods Data of 145 patients with GD and 45 healthy subjects were collected.All cases were detected on the presence of ANA and the level of immunoglobulin,FT3,FT4,and thyroid specific antibodies.Results The presencerate of ANA and the level of IgG in patients with GD were higher than that in healthy controls [(28.28% vs 4.55%);(70.96?26.14 vs 60.41?11.01) mmol/L](P

A Study on the Frequency of the Autoimmune Disorders in Vitiligo Patients

BACKGROUND: The increase of the incidence of autoimmune diseases and the autoimmune pathogenesis of vitiligo were reported. OBJECTIVE: We studied the frequency of autoimmune disorders and positivity of antinuclear antibody in Korean vitiligo patients. METHODS: Vitiligo patients (439 patients) and control subjects (197 patients) were interviewed about their history of autoimmune diseases. Laboratory studies including complete blood cell count, urine analysis, blood chemistry, fasting blood sugar, thyroid function test (T3, free T4, TSH), and antinuclear antibody were performed for the screening of autoimmune disorders. RESULTS: The diseases associated with vitiligo were microcytic hypochromic anemia (3.64%), non-insulin dependent diabetes mellitus (2.96%), thyroid disease (3.96%), atrophic gastritis, and alopecia areata. In the control subjects, the associated diseases were microcytic hypochromic anemia (1.62%), non-insulin dependent diabetes mellitus (4.65%), and thyroid disease (3.49%). These results show that the frequency of autoimmune disorders in vitiligo patients is not significantly higher than that in control subjects. Six (54.5%) out of 11 vitiligo patients with thyroid disease were diagnosed as having thyroid disease for the first time. Four (0.91%) out of 438 vitiligo patients showed positive to antinuclear antibody. Positivity of antinuclear antibody was not higher in vitiligo patients than that in control subjects (1.16%). CONCLUSION: Frequency of autoimmune diseases and positive reaction to antinuclear antibody in vitiligo patients were not significantly higher than those in control subjects.