Epilepsias focales autolimitadas de la infancia / Self-limited Focal Epilepsies of Childhood

Resumen Las Epilepsias Focales Autolimitadas de la Infancia (SELFEs - siglas en inglés) son los síndromes electroclíni cos más prevalentes en edad pediátrica, cuya evolución típica, con inicio y remisión dependientes de la edad, ha permitido que el Grupo de Trabajo de Nosología y Definiciones de la ILAE (2022) las defina como "Epilep sias focales autolimitadas de la infancia", estableciendo así, criterios de alerta y exclusión para estandarizar su diagnóstico. Dentro de estos síndromes se incluyen: la Epilepsia Autolimitada con Espigas Centrotemporales (previamente Epilepsia Rolándica), Epilepsia Autolimi tada con Crisis Autonómicas. (previamente Síndrome de Panayiotopoulos), Epilepsia Visual Occipital Infantil (previamente Síndrome de Gastaut), y Epilepsia Fotosen sible del Lóbulo occipital. Ya no se recomienda utilizar el término "benignas" para referirse a ellas, ya que esto haría caso omiso de las comorbilidades que padecen algunos individuos. Asimismo, el término "idiopático" sólo se utiliza ahora para denominar a los síndromes clasificados como Epilepsias Generalizadas Idiopáticas.

Abstract Self-limited Focal Epilepsies of Childhood (SELFEs) are the most prevalent electroclinical syndromes in pe diatric age, whose typical evolution, with age-dependent onset and remission, has allowed the ILAE Nosology and Definitions Working Group (2022) to define them as "Self-limited Focal Epilepsies of Childhood", thus establishing alert and exclusion criteria to standardize their diagno sis. These syndromes include: Self-limited Epilepsy with Centrotemporal Spikes (previously Rolandic Epilepsy), Self-limited Epilepsy with Autonomic Seizures (previ ously Panayiotopoulos Syndrome), Childhood Occipital Visual Epilepsy, (previously Gastaut Syndrome), and Photosensitive Occipital Lobe Epilepsy. Using the term "benign" to refer to them is no longer recommended, as this would ignore the comorbidities some individuals suffer. Also, the term "idiopathic" is now only used to refer to the syndromes classified as Idiopathic General ized Epilepsies.

Pneumomediastinum secondary to diabetes ketoacidosis in high altitude: a case report / 中国综合临床

Diabetic ketoacidosis (DKA), a serious acute complication of diabetes mellitus, mainly manifests as hyperglycemia, ketosis, and acidosis. It is a metabolic syndrome resulting from insulin deficiency and increased insulin-antagonistic hormone levels. While type 2 diabetes mellitus complicated by DKA is relatively uncommon, secondary pneumomediastinum in DKA is extremely rare. Following alveolar rupture, air can travel through various routes to reach the hilum, causing anterior, middle, or posterior pneumomediastinum or even leading to intracranial epidural pneumatosis. The diagnosis of pneumomediastinum is mainly dependent on chest computed tomography findings. After the successful treatment of DKA, pneumomediastinum usually resolves spontaneously within 5-10 days with a good prognosis. One DKA patient admitted to Dege County People's Hospital developed Kussmaul respirations, followed by an increase in intra-alveolar pressure, an elevation in intra and extra-alveolar pressure difference, and protein decomposition in the alveolus wall, which promoted alveolar rupture and induced mediastinal emphysema. After rapid fluid replacement, blood glucose control with insulin, and maintenance of acid-base balance (correction DKA), the mediastinal emphysema was spontaneously absorbed. Through the analysis of the clinical data of this case, the purpose is to improve the clinicians' internal understanding of the relationship between mediastinal emphysema and DKA, avoid over-examination and over-treatment, and provide strategies for correct diagnosis and treatment.

Exantema intertriginoso y flexural simétrico por fármacos: presentación de un caso / Symmetrical drug-related intertriginous and flexural exanthema: a case report

Symmetrical drug-related intertriginous and flexural exanthema (SDRIFE) is a benign, self-limited, immune-mediated, symmetric erythematous rash involving the buttocks and other intertriginous/flexural areas, observed after systemic exposure to a drug in an individual with or without prior sensitization. We present a 70-year old patient, who presented SDRIFE after the administration of piperacillin-tazobactam which improved rapidly after its suspension.

El exantema intertriginoso y flexural simétrico relacionado con fármacos (SDRIFE, por su sigla en inglés) es una erupción eritematosa simétrica, inmunomediada, benigna y autolimitada, que compromete glúteos y otras áreas intertriginosas, flexurales o ambas, y que se observa luego de la exposición sistémica a un fármaco en un individuo con sensibilización previa o sin ella. Se comenta el caso clínico de un paciente de 70 años de edad, que presentó SDRIFE posterior a la administración de piperacilina-tazobactam y que mejoró rápidamente luego de su suspensión.

Research progress on the correlation between herpangina and hand⁃foot⁃mouth disease / 口腔疾病防治

@# As acute enterovirus⁃induced infections, herpangina(HA) and hand⁃foot⁃mouth disease(HFMD) are simi⁃lar in many aspects. Although these diseases vary with time and region, many studies have shown that the viruses caus⁃ing HA and HFMD are consistent, and there is no notable difference in partial VP1 gene sequences between different vi⁃ruses. HA and HFMD also resemble each other in epidemiological features. Both infections show significant summer⁃time seasonality, have a strong connection with certain environmental conditions and are most prevalent in young chil⁃dren and infants. Herpangina is thought to be a mild disease, defined as vesicular enanthem and then ulcers of the fau⁃ces and soft palate with presentation of feve r, sore throat, and decreased appetite. HFMD, which could lead to severe symptoms, is also characterized by oral ulcers, although they are chiefly on the buccal mucosa and tongue, and typical vesicular rashes, which are most commonly found on the hands, feet, knees and buttocks. While HA is generally be⁃ lieved to be self⁃limited and has a favorable prognosis, HA with certain clinical characteristics, such as diarrhea, vomit⁃ing, limb jitter and sleepiness, can evolve into HFMD, according to some literature in recent years. However, HA is an independent risk factor for HFMD, and severe cases only present with herpes appearing at the isthmus of the fauces at an early stage, which indicates a strong correlation between them. Clinical manifestations of HA should be considered by medical staff to identify potential children with HFMD as early as possible to prevent its further development or transformation.

Atrofodermia lineal de Moulin / Linear atrophoderma of Moulin

Resumen Es una enfermedad poco frecuente, descripta en 1992 por Moulin, que se presenta entre los 6 y los 20 años de edad, caracterizada por bandas hiperpigmentadas atróficas de distribución blaschkoide, localizadas principalmente en tronco, unilaterales, que no son precedidas por inflamación o cambios esclerodérmicos, induración ni adherencias a planos profundos. En general es una afección autolimitada, cuyos tratamientos resultan ineficaces. Presentamos el caso de un niño de 12 años, con lesiones en tronco compatibles con el diagnóstico de Atrofodermia lineal de Moulin (ALM).

Abstract It is a disease not very frequent, described in 1992 by Moulin, which occurs between 6 and 20 years of age, characterized by hyperpigmented atrophic bands distribution blaschokoide, mainly located in trunk, unilateral, that are not preceded by inflammation or changes sclerodermal, induration, or adhesions to deep. It is a self-limited condition, whose treatments are ineffective. We present the case of a boy, 12 years old, with atrophic, hyperpigmented and asymptomatic plates, distributed from the left mammary region to the homolateral back, following the lines of Blaschko. Refers that is started as a hyperpigmented macula and in recent years it has been atrophying, it is not accompanied by any symptomatology, and was not preceded by inflammatory. We performed laboratory test with complete blood count, renal function, liver function and antibody titers, which were normal; and incisional biopsy by punch, that reported, for a sample stained with hematoxylin-eosin, epidermis of variable thickness, canned, no cellular atypia or disorders madurativos. Dermis impresses discreetly thickened, with homogenization of collagen. Slight perivascular inflammatory infiltrate. With clinical and anatomopathology we arrive at the diagnosis of Linear atrophoderma of Moulin.

Evolución atípica del síndrome de Panayiotopoulos a síndrome de punta-ondas continuas durante el sueño lento / Atypical evolution of Panayiotopoulos syndrome to continuous spikes and wave's syndrome during slow sleep

Introducción: Las epilepsias focales idiopáticas constituyen uno de los grupos de epilepsias más frecuentes en la infancia. Excepcionalmente los pacientes con este tipo de epilepsias tienen evoluciones atípicas que constituyen un reto diagnóstico y terapéutico. Objetivo: Ilustrar la evolución atípica de la epilepsia focal idiopática tipo Panayiotopoulos. Presentación del caso: Adolescente de 13 años que presentó su primera crisis epiléptica a los 5 años de edad, de breve duración, mientras dormía tuvo apertura ocular, desviación de los ojos a la izquierda, abundante salivación y presentó un vómito. En tres años tuvo solo tres crisis. No recibió tratamiento con fármacos antiepilépticos hasta después de la tercera crisis, que fue más prolongada. Tras iniciar tratamiento con carbamazepina comenzó a presentar dificultades en el aprendizaje y marcada hiperactividad. Un electroencefalograma interictal de sueño demostró descargas de punta-ondas continuas en el sueño lento. Después de dos años de tratamiento se alcanzó la normalidad en el estudio electroencefalográfico de sueño, con retirada inicial de la carbamazepina, e introducción progresiva de clobazam y valproato de magnesio. Evolutivamente el paciente mantuvo las dificultades en el aprendizaje, con mejoría notable de su hiperactividad, sin recurrencia de crisis epilépticas. Conclusiones: El caso presentado constituye un ejemplo infrecuente de un paciente con una epilepsia focal idiopática con evolución atípica, probablemente inducida por la carbamazepina, con cuadro clínico-electroencefalográfico de más de dos años de duración, con mejoría favorecida por el tratamiento finalmente empleado, la evolución natural del síndrome o el efecto de ambos (AU)

Introduction: Idiopathic focal epilepsies are one of the most frequent epilepsy groups in childhood. Exceptionally, patients with this type of epilepsy have atypical evolutions that constitute a diagnostic and therapeutic challenge. Objective: To illustrate the atypical evolution of idiopathic focal epilepsy, type Panayiotopoulos. Case presentation: A 13-year-old adolescent who presented his first epileptic seizure at 5 years old, of short duration, while he slept had an eye opening, deviation of the eyes to the left, abundant salivation and vomiting. In three years he had only three seizures. He did not receive treatment with antiepileptic drugs until after the third seizure, which was longer. After starting treatment with carbamazepine, he began to have learning difficulties and marked hyperactivity. A sleep's interictal electroencephalogram showed continuous spikes and wave's discharges during the slow sleep. After two years of treatment, the normalization of the sleep electroencephalogram was achieved, with withdrawal of carbamazepine, and progressive introduction of clobazam and magnesium valproate. The patient remained evolutionarily with learning difficulties, with significant improvement in hyperactivity, without recurrence of seizures. Conclusions: The case presented is an infrequent example of a patient with idiopathic focal epilepsy with atypical evolution, probably induced by carbamazepine, with clinical-electroencephalographic symptoms during more than two years, with improvement favored by the final treatment used, the natural evolution of the syndrome or the effect of both(AU)

Dermatitis flagelada inducida por bleomicina: a propósito de un caso / Bleomycin-induced flagellated dermatitis: a case report

INTRODUCCIÓN: La Dermatitis Flagelada es una patología infrecuente, con lesiones cutáneas características, que se desarrolla por el uso de Bleomicina. Clínicamente se presenta como maculas eritematosas o hiperpigmentadas de disposición lineal con patrón flagelar, en tronco y/o extremidades superiores. Presenta evolución autolimitada por lo que su tratamiento varía desde conducta expectante hasta uso de corticoides tópicos u orales. OBJETIVO: Presentación de un caso clínico de Dermatitis flagelada secundaria a Bleomicina en paciente pediátrico con antecedentes de neoplasia de sistema nervioso central. CASO CLÍNICO: Escolar de 8 años, sexo femenino, con antecedentes de tumor prima rio de células germinales mixto intracraneal (selar y supraselar) y panhipopituitarismo secundario. Recibe tratamiento quimioterapéutico según protocolo PEB, con uso de Bleomicina EV por 3 días. A los 2 días posteriores, inicia prurito intermitente, asociado a máculas eritematosas y pigmentadas de distribución lineal, siguiendo patrón flagelado, con aislados signos de excoriación, en región abdominal y dorso alto. Se indica tratamiento tópico con corticoides de moderada potencia por 10 días, con respuesta clínica satisfactoria. CONCLUSIONES: Se debe tener una alta sospecha diagnóstica en pacientes pediátricos con historia de administración previa del fármaco y aparición de lesiones cutáneas características, lo que permitirá una conducta adecuada respecto a su manejo y a la continuidad de la quimioterapia.

INTRODUCTION: Flagellated dermatitis is an infrequent pathology, with characteristic skin lesions, which is developed due to the use of bleomycin. Clinically it occurs as erythematous or hyperpigmented maculae of linear disposition with flagellar pattern, in trunk and/or upper extremities. It presents self-limited evolution, therefore, its treatment varies from expectant management to the use of topical or oral corticosteroids. OBJECTIVE: Presentation of a clinical case of flagellated dermatitis secondary to bleomycin in a pediatric patient with history of central nervous system neoplasia. CLINICAL CASE: 8 years, schoolchild, female, with a history of primary intracranial mixed germ cell tumor (sellar and suprasellar) and secondary panhypopituitarism. She receives chemotherapeutic treatment according to the PEB protocol, with use of IV bleomycin during three days. After two days, intermittent pruritus begins, associated with erythematous and pigmented maculae of linear distribution, followed by a flagellated pattern, with isolated signs of excoriation, in the abdominal region and upper back. Topical treatment with mild potency corticosteroids is indicated for ten days, with a satisfactory clinical response. CONCLUSIONS: There should be a high diagnostic suspi cion in pediatric patients with a history of prior administration of the drug and the appearance of characteristic skin lesions, which will allow adequate behavior regarding its management and the continuity of chemotherapy.

Kikuchi-Fujimoto Disease Case Report.

Kikuchi-Fujintoto disease (KFD) was first reported by 2 Japanese pathologists, Kikuchi and Fujimoto, independently in 1972. KFD is an idiopathic, self-limited necrotizing lymphadenitis. The most common clinical manifestation is cervical lymphadenopathy accompanied by fever, myalgia, leukopenia, and skin rash. The purpose of this paper is to report the first case of Kikuchi-Fujimoto disease in a twelve year old girl in Dr.Cipto Mangunkusumo Hospital. Jakarta.

Comparison of Cell Proliferation between Chronic Ulcerative Colitisand Acute Self-limited Colitis

PURPOSE: The ulcerative colitis is a major chronic intestinal disease of unknown etiology affecting principally the rectum and left colon, and its incidence is markedly increasing in Korea. The main differential diagnosis of ulcerative colitis is an acute self-limited colitis. This study was performed to evaluate difference of cell kinetics between chronic ulcerative colitis and acute self-limited colitis. METHODS: The normal colon (n=25), acute self-limited colitis (n=25) and chronic ulcerative colitis (n=25) were investigated by using Ki-67 immunohistochemical staining for proliferation and TUNEL method for apoptosis. The Ki-67 labeling indices and TUNEL labeling indices were determined. RESULTS: The means of Ki-67 labeling indices in normal colon, acute self-limited colitis and chronic ulcerative colitis were 5.14 5.25%, 6.81 5.73%, 13.10 10.15%, respectively. And the means of TUNEL labeling indices in normal colon, acute self-limited colitis and chronic ulcerative colitis were 1.59 0.10%, 2.54 1.60%, 2.51 1.40%, respectively. CONCLUSIONS: The apoptosis is one of method of cell loss in both acute self-limited colitis and chronic ulcerative colitis. High proliferative activity of chronic ulcerative colitis may predispose to mutational events in colonic mucosa, therefore may be one of the increased cancer risk factors in chronic ulcerative colitis.