Hemangioma infantil. Reporte de un caso de presentacion clinica anular

Reportamos el caso clínico de un lactante menor con una presentación anular de hemangioma, que nos plantea dudas en su clasificación. Se precisan los diagnósticos diferenciales y la necesidad de la determinación del marcador GLUT 1 en hospitales públicos.

We report the clinical case of a young infant with an annular presentation of hemangioma, which raises doubts regarding its classification. Differential diagnoses and the need to determine the GLUT 1 marker in public hospitals are specified.

Hemangioma infantil. Reporte de un caso de presentacion clinica anular / Infantile hemangioma. Report of a case of annular clinical presentation

Reportamos el caso clínico de un lactante menor con una presentación anular de hemangioma, que nos plantea dudas en su clasificación. Se precisan los diagnósticos diferenciales y la necesidad de la determinación del marcador GLUT 1 en hospitales públicos.

We report the clinical case of a young infant with an annular presentation of hemangioma, which raises doubts regarding its classification. Differential diagnoses and the need to determine the GLUT 1 marker in public hospitals are specified.

Atenolol oral comparado con propranolol oral para hemangioma infantil / Oral atenolol compared to oral propranolol for infantile hemangioma

Introducción El hemangioma infantil corresponde al tumor vascular benigno más frecuente de la infancia, con una incidencia de 3 a 10%. Entre los pacientes que requieren tratamiento el uso oral de propranolol, un betabloqueador no selectivo de tipo lipofílico, es usualmente considerado como la terapia de elección. Sin embargo, su uso se ha asociado a diversos efectos adversos, relacionados con su acción ß-2, y a su capacidad de cruzar la barrera hematoencefálica. Debido a esto, el uso oral de atenolol, un betabloqueador selectivo de receptores ß-1, de tipo hidrofílico, podría representar una alternativa válida de tratamiento. Sin embargo, aún existe controversia en relación con la eficacia y seguridad del tratamiento con atenolol como monoterapia, en comparación con el uso de propranolol como monoterapia para esta condición. Métodos Se realizó una búsqueda en Epistemonikos, la mayor base de datos de revisiones sistemáticas en salud, la cual es mantenida mediante el tamizaje de múltiples fuentes de información, incluyendo MEDLINE/PubMed, EMBASE, Cochrane, entre otras. Se extrajeron los datos desde las revisiones identificadas, se analizaron los datos de los estudios primarios, se realizó un metanálisis y se preparó una tabla de resumen de los resultados utilizando el método , GRADE. Resultados Se identificaron nueve revisiones sistemáticas, que en conjunto incluyeron 10 estudios primarios y tres ensayos aleatorizados. Se incluyeron los tres ensayos aleatorizados en el análisis del presente trabajo. Conclusiones El uso de atenolol oral como monoterapia, comparado con el uso de propranolol oral como monoterapia, podría resultar en poca o nula diferencia en cuanto a la probabilidad de remisión completa, la disminución del , la probabilidad de recaída posterior al tratamiento y el riesgo de presentar efectos adversos y efectos adversos severos, en el hemangioma infantil (certeza de la evidencia baja).

Introduction Infantile hemangioma is the most frequent benign vascular tumor in childhood, with an incidence of 3 to 10%. When patients require treatment, oral propranolol, a non-selective lipophilic beta-blocker, is usually considered the therapy of choice. However, its use has been associated with several adverse events related to its ß-2 action and its ability to cross the blood-brain barrier. Because of this, oral atenolol, a hydrophilic ß-1 receptor-selective beta-blocker, may represent a valid treatment alternative. Nonetheless, there is still controversy regarding the efficacy and safety of atenolol when compared with propranolol as monotherapy for this condition. Methods We searched Epistemonikos, the largest database of systematic reviews in health science, which is maintained by screening multiple sources of information, including MEDLINE/PubMed, EMBASE, and Cochrane, among others. Data were extracted from the identified reviews, data from the primary studies were analyzed, a meta-analysis was performed, and a summary table of the results was prepared using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) method. Results Nine systematic reviews were identified, including 10 primary studies and three randomized trials. The three randomized trials were included in the analysis of this investigation. Conclusion The use of oral atenolol compared with oral propranolol as monotherapies may result in little or no difference in terms of likelihood of complete remission, decrease in Hemangioma Activity Score, likelihood of post-treatment relapse, and risk of adverse events and severe adverse events, in infantile hemangioma (low certainty of evidence).

Infantile (congenital) anaplastic intracranial solitary fibrous tumor/hemangiopericytoma—A case report with brief literature review

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a rare primary central nervous system (CNS) tumor, included in the World Health Organization (WHO) 2016 classification. Very few cases have been described in the literature so far, especially the infantile type. It is a mesenchymal tumor of the fibroblastic type, characterized by the fusion of NAB 2 and STAT 6 genes. A 10-month-old boy presented to our neurosurgery department with complaints of increasing head circumference since 1 month of age. The magnetic resonance imaging (MRI) showed a space-occupying lesion measuring 8.2 cm × 7 cm × 6.9 cm in the fronto-temporo-parietal region with a clinical diagnosis of glioma/atypical teratoid rhabdoid tumor (ATRT). The microscopy revealed a spindle cell tumor arranged in a patternless pattern with variable cellularity, increased mitosis, and areas of coagulative necrosis. The immunohistochemistry showed vimentin, CD 34, STAT6, CD99 positivity whereas Glial fibrillary acidic protein, Epithelial membrane antigen, and S-100 negativity. Hence, a diagnosis of anaplastic SFT/HPC (grade-III) was rendered. The patient improved after gross total resection (GTR). The primary intracranial congenital SFT/HPC are extremely rare, often a clinico-radiologically misdiagnosed entity. Thus, the immunohistochemistry/molecular study in addition to histology is mandatory for accurate diagnosis.

Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants / Consenso de especialistas brasileiros sobre o tratamento da síndrome dos espasmos infantis em lactentes

Abstract Background Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms may not respond to most antiseizure medication, and relapse is frequent. Objective To evaluate the literature regarding treatment of IESS and provide a practical guidance to a healthcare system with limited resources. Methods An expert committee from the Brazilian Society of Child Neurology reviewed and discussed relevant scientific evidence in the treatment of IESS regarding the drugs available in Brazil. Results Oral prednisolone and vigabatrin are the most common drugs used as first-line therapy; they are efficient and affordable therapy as both are available in the Brazilian unified health system (SUS, in the Portuguese acronym). Intramuscular adrenocorticotropic hormone (ACTH) presents similar efficacy as oral prednisolone but has a higher cost and is not available in Brazil. Other antiseizure medications such as topiramate, levetiracetam, or benzodiazepines have limited response and are prescribed as adjuvant therapy. If the health service has nutritionists, a ketogenic diet should be implemented for those not responding to hormonal and vigabatrin treatment. Epilepsy surgery is mainly indicated for patients with focal lesions that do not respond to pharmacological therapy. Conclusion Early treatment of IESS with efficient drugs is feasible in our country. Using standard protocols increases the odds of achieving complete cessation in a shorter time and decreases relapse.

Resumo Antecedentes A síndrome do espasmo epiléptico infantil (IESS) é uma condição rara, mas grave, que afeta crianças precocemente e geralmente é secundária a um distúrbio cerebral identificável, estando relacionada a deterioração psicomotora na infância e a epilepsia na vida adulta. O tratamento é desafiador, pois os espasmos infantis podem não responder à maioria dos medicamentos anticrises e as recidivas são frequentes. Objetivo Avaliar a literatura sobre o tratamento de IESS e fornecer uma orientação prática para um sistema de saúde com recursos limitados. Métodos Um comitê de especialistas da Sociedade Brasileira de Neurologia Infantil revisou e discutiu evidências científicas relevantes no tratamento da IESS em relação aos medicamentos disponíveis no Brasil. Resultados Prednisolona oral e vigabatrina são os fármacos mais comumente usados como terapia de primeira linha; são eficientes e acessíveis, já que ambos estão disponíveis no sistema único de saúde brasileiro (SUS). O ACTH intramuscular apresenta eficácia semelhante à prednisolona oral, mas tem custo mais elevado e não está disponível no Brasil. Outros medicamentos anticonvulsivos, como topiramato, levetiracetam ou benzodiazepínicos, têm resposta limitada e são prescritos como terapia adjuvante. Se o serviço de saúde tiver nutricionista, deve-se implementar dieta cetogênica para aqueles que não respondem ao tratamento hormonal e vigabatrina. A cirurgia de epilepsia é indicada principalmente para pacientes com lesões focais que não respondem à terapia farmacológica. Conclusão O tratamento precoce da IESS com fármacos eficazes é factível em nosso meio. O uso de protocolos padronizados aumenta as chances de alcançar a cessação completa em um tempo menor e diminui a recaída.

COVID-19 in Children with West Syndrome: An Ambispective Study

Objectives To study the course of West syndrome (WS) and coronavirus disease-19 (COVID-19) in children with WS who contracted SARS-CoV-2 infection. Methods This ambispective study was conducted at a tertiary-care center in North India between December 2020 and August 2021 after approval from the Institute Ethics Committee. Five children with WS, positive for COVID-19 based on RT-PCR, fulflled the inclusion criteria. Results One child with COVID-19 during the frst wave was retrospectively included while four children (of the 70 children screened) were prospectively enrolled. The median age at onset of epileptic spasms was 7 mo (2 boys), and that at presentation with COVID-19 was 18.5 mo. Three had underlying acquired structural etiology. Three were in remission following standard therapy, while two had ongoing spasms at the time of COVID-19 illness. During the illness, two of those in remission continued to be in remission while one child had a relapse. The children with ongoing epileptic spasms had variable course [one had persistent spasms and other had transient cessation lasting 3 wk from day 2 of COVID-19 illness, but electroencephalography (on day 8 of COVID-19 illness) continued to show hypsarrhythmia]. Fever was the most typical symptom (and sometimes the only symptom) of COVID-19, with a duration ranging from 1–8 d. Two children had moderate COVID-19 illness requiring hospitalization, while the rest had a mild illness. All the afected children had complete recovery from COVID-19. Conclusion The severity of COVID-19 illness in children with WS is often mild, while the subsequent course of WS is variable.

Occurrence and subsequent development of vertical deviations in patients treated surgically for infantile esotropia

Purpose: The purpose of this study was to evaluate the onset of dissociated vertical deviation (DVD) and inferior oblique overaction (IOOA), their subsequent development, and their correlation with pre and postoperative parameters. Methods: Medical records of patients with infantile esotropia who underwent surgery between 2005 and 2017 were retrospectively reviewed. DVD and IOOA were measured before and after surgery. Patients were divided into two groups based on horizontal and vertical deviation at the time of presentation: those with infantile esotropia only (group A) and patients with infantile esotropia who developed vertical deviation (group B). Results: Out of a total of 102 patients, DVD occurrence was seen in 53 patients (51.9%) and IOOA was seen in 50 patients (48.04%). DVD was seen in 22 patients at the time of initial examination and in 31 patients postoperatively. IOOA at presentation was seen in 45 patients (44.1%) and 5 patients (8.8%) postoperatively. No statistical difference was found in the age of surgery, angle of deviation, mean follow?up, and mean refractive error within both groups. The postoperative motor outcome was statistically comparable between the two groups (P = 0.29). Sensory outcomes of fusion (P = 0.048) and stereopsis (P?value = 0.00063) were better in group A. Conclusion: No correlation was found between the age of occurrence and development of vertical deviation with refractive error, angle of deviation, age, or type of surgery. We found that motor outcomes are not affected but sensory outcomes are affected in patients with vertical deviations. This indicates that DVD and IOOA are developed due to inherent disruption of fusion and stereopsis

Hemangioma segmentario de la barba / Segmental Beard Hemangioma

Los hemangiomas infantiles son el resultado de la proliferación de células del endotelio vascular y representan los tumores benignos más frecuentes en la infancia, con una incidencia estimada del 4-10% en bebés caucásicos. Se clasifican según el número, la profundidad y la distribución. Dentro de esta última clasificación se encuentran aquellos denominados segmentarios, que se caracterizan por su distribución extensa en áreas de prolongaciones mesodérmicas embrionarias. Se comunica el caso de una paciente evaluada al mes y medio de vida, con un hemangioma extenso del área mandibular y cuello anterior (hemangioma segmentario de la barba). Se describe la importancia de los estudios complementarios para evaluar el compromiso de órganos subyacentes, para detectar síndromes asociados y definir el tratamiento sobre la base de estos resultados. (AU)

Infantile hemangiomas arise from the proliferation of vascular endothelial cells and represent the most common benign tumors in infancy, with an estimated incidence of 4-10% in Caucasian infants. They vary according to their number, depth, and distribution. Within the latter classification are the so-called segmental ones, which feature an extensive distribution in areas of embryonic mesodermal extensions. We report the case of a patient evaluated at one and a half months of life with an extensive hemangioma of the mandibular area and anterior neck (segmental hemangioma of the beard). We describe the importance of complementary studies for evaluating the involvement of underlying organs, detecting associated syndromes, and defining the treatment based on these findings. (AU)

Abdominoscrotal hydrocele with ipsilateral hydroureteronephrosis in an adult: A case report

Abdominoscrotal hydrocele (ASH) is very rare in adults and difficult to suspect on clinical examination. ASH is a very unusual presentation of scrotal hydrocele with extension intra-abdominally through the inguinal canal either communicating to the peritoneal cavity or non-communicating. An ASH is a congenital pathology involving a scrotal hydrocele expanding through the inguinal canal and reaching the abdominal cavity. Here, we present the case of a 25-year-old man admitted with a complaint of pain and lump in the left lower abdomen for 2 months and swelling in the left groin for 2 years. Ultrasound and computed tomography suggest left ASH with left-sided hydroureteronephrosis. A left DJ stent was placed and on exploration, a large cystic mass was seen intraperitoneally extending into the scrotum through the inguinal canal. Excision of the abdominal part and partial excision of the scrotal part were done with eversion of the remaining tunica vaginalis sac. Although ASH is a rare entity, it should always be kept on the list of differential diagnoses during the complaint of large abdominal mass in adults.

Impacto del aislamiento social por COVID-19 en niños y niñas con trastornos del espectro autista y sus familias en la provincia de Misiones / Impact of social isolation by COVID-19 on children with autism spectrum disorders and their families in the province of Misiones

RESUMEN INTRODUCCIÓN El trastorno del espectro autista (TEA) se caracteriza por alteraciones en la comunicación y patrones restrictivos del comportamiento. El Aislamiento Social, Preventivo y Obligatorio (ASPO) en Argentina generó cambios en las dinámicas familiares de personas con TEA. El objetivo del estudio fue describir el impacto del ASPO en el acompañamiento de niños/as con TEA. MÉTODOS Se creó un instrumento específico de diseño mixto, con implementación de encuestas virtuales a familiares de personas con TEA. RESULTADOS Las 66 encuestas obtenidas revelaron ventajas, tales como la posibilidad de compartir tiempo en familia y conocer las necesidades de las/os hijas/os, el establecimiento de un ambiente con estímulos adecuados y ausencia de distractores, y la enseñanza con ritmos de aprendizaje y contenidos ajustados a las necesidades de niños/as con TEA. También se encontraron dificultades para la socialización: surgimiento de comportamientos no adaptativos (crisis, ansiedad, agresividad), barreras en la comunicación entre docentes y familias, imposibilidad de acceder a terapias y obstáculos para acceder a terapias virtuales. DISCUSIÓN El ASPO incidió en la vida cotidiana de las personas con TEA, de manera similar a lo observado en otras latitudes. A partir de lo experimentado durante el ASPO se proponen estrategias contextualizadas a la situación de pandemia actual y a potenciales escenarios similares.

ABSTRACT INTRODUCTION Autism spectrum disorder (ASD) is characterized by alterations in communication and restrictive behavior patterns. The Social, Preventive and Compulsory Isolation in Argentina changed family dynamics of people with ASD. The objective of this study was to describe the impact of isolation on accompanying children with ASD. METHODS A specific instrument of mixed design was created by conducting virtual surveys to relatives of ASD children. RESULTS The 66 surveys included in the study revealed advantages, such as the possibility of sharing family time and recognizing children's needs, environment with adequate stimuli and without distractors as well as teaching according to learning pace and content adjusted to ASD children. They also found difficulties in socialization: emergence of non-adaptive behaviors (crisis, anxiety, aggressiveness), communication barriers between teachers and families, therapy inaccessibility and obstacles to access to online therapies. DISCUSSION The isolation affected ASD people's daily life, similarly as in other countries. Based on the experiences during the isolation, strategies are proposed within the framework of the current pandemic situation and for potential similar scenarios.

Persistence of traumatic infantile hemiparesis in an adult - A rare case report

Hemiplegia is the physical manifestation of an injury to a specific area of the brain that controls motor function. Hemiplegia may develop suddenly, or evolve over days, weeks or months. Hemiparesis/ hemiplegia is rare in children. It causes significant mortality and morbidity. Infections are an important cause of neurological deficit, in the developing countries. Once the injury has occurred, the symptoms should not worsen. However, because of lack of mobility, other complications can occur. Complications may include muscle and joint stiffness, and shortening of limbs. The term acute infantile hemiplegia denotes certain cases of hemiplegia of sudden onset in children, of a few weeks to 6 or more years of age. The outcome may be fatal; or there may be permanent hemiplegia with mental impairment. There may be slow partial recovery; or rapid and complete recovery. We herewith present a 35 old male, who presented with seizures. 15 years ago he sustained head injury as he fell down from a tree. He was diagnosed with subdural hematoma and was evacuated through craniotomy, after which he developed residual right hemiparesis. The right hemiparesisis was still persisting as residual hemiparesis on right side with shortening of limbs. A diagnosis of Post traumatic Infantile hemiparesis on right side was made.

Síndrome de Aicardi: revisión bibliográfica

Introducción: el síndrome de Aicardi (SA; OMIM #304050) es un trastorno genético raro, cuya incidencia es de aproximadamente 1/100.000. Fue descrito en 1965 como una triada consistente en agenesia del cuerpo calloso, lagunas coriorretinianas y espasmos infantiles. Asocia discapacidad intelectual severa y epilepsia de difícil control. Aunque su espectro clínico es variable, tiene por lo general un pronóstico infausto debido a la elevada morbimortalidad asociada. Se considera un trastorno esporádico causado por variantes patogénicas en heterocigosis de un gen ligado al cromosoma X, que causa mortalidad embrionaria en varones hemicigotos. Objetivo: este trabajo pretende llevar a cabo una revisión bibliográfica acerca de la literatura científica disponible del síndrome de Aicardi. De esta manera se hará una actualización sobre esta entidad en cuanto a definiciones, prevalencia e incidencia, etiología, espectro clínico y pronóstico de los pacientes afectos. Materiales y métodos: se lleva a cabo una búsqueda bibliográfica retrospectiva en las principales bases de datos científicas. Para ello, se utilizan las palabras clave "Aicardi", "agenesia del cuerpo calloso", "espasmos infantiles" y "encefalopatía epiléptica". Conclusiones: desde su descripción se ha ido ampliando el espectro de manifestaciones clínicas del síndrome. Actualmente no se conoce la existencia de un biomarcador que posibilite el diagnóstico, por lo que éste continúa siendo eminentemente clínico. Se debe tener un alto nivel de sospecha en espasmos infantiles de debut precoz en mujeres con alteraciones en neuroimagen.

Introduction: Aicardi syndrome (AS; OMIM #304050) is a rare genetic disorder, with an incidence of approximately 1/100,000. It was described in 1965 as a triad consisting of agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. It is associated with severe intellectual disability and difficult-to-control epilepsy. Although its clinical spectrum is variable, it generally has a poor prognosis due to the associated morbidity and mortality. It is considered a sporadic disorder caused by heterozygous pathogenic variants of a gene linked to the X chromosome, which causes embryonic mortality in hemizygous males. Objective: this article performs a bibliographic review of the available scientific literature on Aicardi syndrome. In doing so, we hope to update the disorder's definitions, prevalence and incidence, etiology, clinical spectrum and prognosis of affected patients. Materials and methods: we performed a retrospective bibliographic search in the main scientific databases. For this, we searched for the keywords "Aicardi", "agenesia of the corpus callosum", "infantile spasms" and "epileptic encephalopathy". Conclusions: since it was first described, the spectrum of clinical manifestations of the syndrome has been expanding. Currently, there is no known biomarker that makes diagnosis possible, so it continues to be eminently clinical diagnosis. A high level of suspicion should be present in cases of early-onset infantile spasms in women with neuroimaging abnormalities.

Ultrasound techniques for the detection of developmental dysplasia of the hip: a systematic review and meta-analysis

ABSTRACT BACKGROUND: Developmental dysplasia of the hip (DDH) encompasses a broad spectrum of hip pathologies, including femoral or acetabular dysplasia, hip instability, or both. According to the medical literature, ultrasonography is the most reliable diagnostic method for DDH. Several techniques for the assessment of hips in newborns and infants, using ultrasonography, have been described. OBJECTIVE: To compare the accuracy of the Graf technique and other diagnostic techniques for DDH. DESIGN AND SETTING: A systematic review of studies that analyzed ultrasound techniques for the diagnosis of DDH within an evidence-based health program of a federal university in São Paulo (SP), Brazil. METHODS: A systematic search of relevant literature was conducted in the PubMed, EMBASE, Cochrane Library, CINAHL, and LILACS databases for articles published up to May 5, 2020, relating to studies evaluating the diagnostic accuracy of different ultrasound techniques for diagnosing DDH. The QUADAS 2 tool was used for methodological quality evaluation. RESULTS: All hips were analyzed using the Graf method as a reference standard. The Morin technique had the highest rate of sensitivity, at 81.12-89.47%. The Suzuki and Stress tests showed 100% specificity. The Harcke technique showed a sensibility of 18.21% and specificity of 99.32%. CONCLUSION: All the techniques demonstrated at least one rate (sensibility and specificity) lower than 90.00% when compared to the Graf method. The Morin technique, as evaluated in this systematic review, is recommended after the Graf method because it has the highest sensitivity, especially with the three-pattern classification of 89.47%. REGISTRATION NUMBER: Identifier: CRD42020189686 at the International Prospective Register of Systematic Reviews (identifier: CRD42020189686).

Anatomical and surgical considerations and outcomes in infantile Terson syndrome

Purpose: To study the presentation and outcomes of infantile Terson syndrome (TS). Methods: This was a retrospective analysis of 18 eyes of nine infants diagnosed to have TS?related intraocular hemorrhage (IOH). Results: Nine infants (seven males) were diagnosed to have IOH secondary to TS, of which eight infants had imaging features suggestive of intracranial bleed meeting our definite criteria. Median age at presentation was 5 months. In 11 eyes of six infants with suspected birth trauma, the median age of presentation was 4.5 months (range 1–5 months) of which one baby had a history of suction cup?aided delivery and four babies had a history of seizures. Vitreous hemorrhage (VH) was noted in 15 eyes (extensive in 11 eyes). Ten of these eyes showed membranous vitreous echoes, or triangular hyperechoic space with apex at the optic nerve head (ONH) posteriorly and base at the posterior lens capsule anteriorly, with or without dot echoes in the rest of the vitreous cavity, with a configuration of “tornado?like hemorrhage” suggestive of Cloquet’s canal hemorrhage (CCH). Eight eyes underwent lens?sparing vitrectomy (LSV) and one eye underwent lensectomy with vitrectomy (LV). On follow?up, disc pallor and retinal atrophy were noted in 11 and 10 eyes, respectively. The mean follow?up was 62 months (1.5 month–16 years). Visual acuity/behavior improved in all cases at the final follow?up. Developmental delay was noted in four children. Conclusion: Unexplained and altered vitreous hemorrhage with typical ultrasonography (USG) features should raise the suspicion of CCH in TS. Despite early intervention to clear visual axis, anatomical and visual behavior may remain subnormal.

Correlation between serum zinc and brain injury in infants with mild gastroenteritis complicated with benign infantile convulsion and febrile convulsion / 中华危重病急救医学

Objective:To investigate the association between serum zinc levels and convulsive brain injury in infants with mild gastroenteritis complicated with benign infantile seizures (BICE) and febrile seizures (FC).Methods:A case-control study method was conducted. 120 children with mild gastroenteritis and convulsion admitted to the First Affiliated Hospital of Hebei North University from January 2020 to January 2022 were enrolled as the research subjects. They were divided into BICE group and FC group according to the type of convulsion. The serum zinc level, the frequency and duration of convulsion, and the occurrence of convulsive brain injury in the two groups were recorded. Multivariate Logistic regression analysis was used to screen the risk factors for convulsive brain injury. The Spearman correlation method was used to analyze the association between serum zinc levels, clinical characteristics of convulsion and convulsive brain injury.Results:A total of 120 children were enrolled, of which 81 developed to BICE and 39 developed to FC during hospitalization. The serum zinc level of children in the FC group was significantly lower than that in the BICE group (μmol/L: 39.24±6.50 vs. 48.65±7.21, P < 0.01). In the BICE group and FC group, the serum zinc level in children with more than 2 convulsions was significantly lower than that in the children with one convulsion (μmol/L: 37.65±6.50 vs. 53.17±7.55 in the BICE group, and 30.27±5.58 vs. 44.16±7.57 in the FC group, both P < 0.01). Serum zinc level in children with convulsion duration ≥5 minutes was significantly lower than that in the children with convulsion duration < 5 minutes (μmol/L: 38.75±6.74 vs. 51.21±7.58 in the BICE group, and 31.08±5.46 vs. 45.19±7.25 in the FC group, both P < 0.01). Moreover, the serum zinc level of children with different convulsion frequency and duration in the FC group was significantly lower than that in the BICE group (all P < 0.01). Among the 120 children, 9 cases of convulsive brain injury occurred, and the incidence rate was 7.50%. The incidence of convulsive brain injury in the BICE group was 1.23% (1/81), which was significantly lower than 20.51% in the FC group (8/39, P < 0.01). The serum zinc level of children with convulsive brain injury was significantly lower than that of children with non-brain injury (μmol/L: 28.50±5.00 vs. 60.22±7.31, P < 0.01), and the number of convulsion was significantly higher than that of non-cerebral injury (≥ 2 convulsions: 100.00% vs. 1.80%, P < 0.01), and the duration of convulsion in children with brain injury was significantly longer than that of non-brain-injured children (convulsion duration ≥5 minutes: 100.00% vs. 11.71%, P < 0.01). Multivariate Logistic regression analysis showed that decreased serum zinc level [odds ratio ( OR) = 2.147, 95% confidence interval (95% CI) was 1.354-3.403], increased number of convulsion ( OR = 3.452, 95% CI was 1.266-9.417), and prolonged convulsion duration ( OR = 3.117, 95% CI was 1.326-7.327) were independent risk factor for convulsive brain injury in children with mild gastroenteritis and convulsion (all P < 0.05). Spearman correlation analysis showed that serum zinc level, convulsion ≥2 times, duration of convulsion ≥5 minutes and convulsion ≥2 times + convulsion duration ≥5 minutes were significantly negatively correlated with the occurrence of convulsive brain injury in FC children ( r values were -0.546, -0.517, -0.522, and -0.528, all P < 0.01). There was no significant correlation between serum zinc level, convulsion ≥2 times, convulsion duration ≥5 minutes and convulsion ≥2 times+convulsion duration ≥5 minutes and convulsive brain injury in BICE children ( r values were -0.281, -0.129, -0.201, -0.243, all P > 0.05). Conclusions:Serum zinc level is related to the characteristics of convulsive symptoms in children with mild gastroenteritis complicated with FC, and has a strong negative correlation with the occurrence of convulsive brain injury. Active targeted intervention and treatment may help reduce the incidence of brain injury in children.

Clinical characteristics of oral Propranolol in the treatment of 4 patients with PHACES syndrome and literature review / 中华实用儿科临床杂志

Objective:To summarize the clinical data and imaging characteristics of patients with PHACES syndrome treated with oral Propranolol.Methods:The clinical data of 4 cases of PHACES syndrome treated with oral Propranolol in Children′s Hospital, Capital Institute of Pediatrics from October 2018 to October 2022 were retrospectively analyzed.Relevant studies reporting the treatment of PHACES with Propranolol were retrieved in PubMed, China National Knowledge Infrastructure (CNKI) and Wanfang Data.Results:Three cases of the 4 patients with PHACES syndrome treated with Propranolol obtained relieved symptoms of facial hemangioma, and 1 case died due to late treatment, complication of severe cardiovascular malformation, and treatment abandonment by parents.A total of 7 clinical studies on the use of Propranolol in the treatment of PHACES were retrieved, including 6 retrospective studies and 1 observational study.Most studies have shown that Propranolol is well tolerated in the treatment of PHACES syndrome, and most of cases have relieved facial hemangioma.The main factors affecting the prognosis are the degree of damage to middle and small arteries such at brain, aorta, chest and neck.Propranolol treatment can improve the prognosis.Conclusions:Oral Propranolol is currently the first-line treatment for PHACES syndrome, and most patients tolerate oral Propranolol well.

Prognosis and influencing factors of infantile spasms / 中华实用儿科临床杂志

Infantile spasms (IS) is one of the most prevalent epileptic encephalopathies in infants and toddlers.The primary clinical features are episodes of spastic spasms, and an electroencephalogram may exhibit hypsarrhythmia features.The majority of patients have a poor prognosis, exhibiting uncontrolled seizures, conversion to other seizure types, as well as intellectual and motor developmental impairments.With the advancement of neurosurgery, genetics, and neuroimaging, various studies have been conducted on the etiologies and pathogenesis of IS; as a result, the prognosis of IS has substantially improved.This article introduced the prognosis of IS and the contributing factors affecting the prognosis.

Progress in surgical treatment of infantile spasms / 中华实用儿科临床杂志

Infantile spasms(IS) is the prevalent epilepsy syndrome in infancy, characterized by an early age of onset, distinctive seizure type, hypsarrhythmia on electroencephalography, and psychomotor retardation.Despite the fact that medication therapy is the primary treatment for IS, there are still some children with IS who are resistant to drug therapy, which is extremely detrimental to their prognosis.Therefore, surgical therapy has increasingly become one of the most prominent IS treatments.As of now, the indications, preoperative evaluation, and surgical techniques of surgery are continuously being investigated based on variables such as the disease, etiology, age, etcetera.The effect of surgery on cognitive function, physical function, linguistic capacity, and memory function is gaining increasing interest.This article described the indications, preoperative evaluation, surgical procedures (including resection, palliative surgery, and stereotactic surgery), and postoperative efficacy of surgical treatment for IS, with the goal of improving the prognosis of IS through precise surgical treatment, and providing more treatment options and a good long-term prognosis for children with drug-resistant IS.

Pharmacological treatments for children with infantile spasms / 中华实用儿科临床杂志

Infantile spasms is the most prevalent epileptic encephalopathy, and its prognosis is often poor.The fundamentals of treatment include early diagnosis and intervention, as well as long-term rehabilitation management.Pharmaceutical treatment is regarded as the primary and most important treatment for infantile spasms.Most importantly, it is based upon a standardized drug selection and administration.This article outlined the clinical application and progress of pharmacological therapy for the infantile spasms.

Newest etiology of infantile spasms syndrome / 中华实用儿科临床杂志

Infantile spasms syndrome (ISs) is a kind of catastrophic epileptic encephalopathy.Epileptic spasms originating in infancy or early childhood, typically accompanied by an electroencephalographic pattern of hypsarrhythmia, and developmental regression are common clinical manifestations.ISs prognosis is primarily determined by its etiology.In recent years, the rapid development of neuroimaging and genetic detection technology has greatly enriched the etiological spectrum of ISs, especially the genetic-related etiology.Likewise, the etiological categorization of ISs has gotten increasingly specific in response to clinical demands.Despite these advances, the etiology of ISs remains complicated and variable, with around one-third of children having unidentified causes.Consequently, more detection methods and studies are still needed to identify other potential etiologies.The etiologic categorization of ISs will be evaluated in this article.