COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report.

BACKGROUND: The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of immunity (IEI). Not much is known about vaccine safety in IEI, and whether vaccination attenuates infection severity in IEI. OBJECTIVE: To estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI. METHODS: We built a secure registry database in conjunction with the US Immunodeficiency Network to examine vaccination frequency and indicators of safety and effectiveness in IEI patients. The registry opened on January 1, 2022, and closed on August 19, 2022. RESULTS: Physicians entered data on 1245 patients from 24 countries. The most common diagnoses were antibody deficiencies (63.7%). At least one COVID-19 vaccine was administered to 806 patients (64.7%), and 216 patients received vaccination prior to the development of COVID-19. The most common vaccines administered were mRNA-based (84.0%). Seventeen patients were reported to seek outpatient clinic or emergency room care for a vaccine-related complication, and one patient was hospitalized for symptomatic anemia. Eight hundred twenty-three patients (66.1%) experienced COVID-19 infection. Of these, 156 patients required hospitalization (19.0%), 47 required ICU care (5.7%), and 28 died (3.4%). Rates of hospitalization (9.3% versus 24.4%, p < 0.001), ICU admission (2.8% versus 7.6%, p = 0.013), and death (2.3% versus 4.3%, p = 0.202) in patients who had COVID-19 were lower in patients who received vaccination prior to infection. In adjusted logistic regression analysis, not having at least one COVID-19 vaccine significantly increased the odds of hospitalization and ICU admission. CONCLUSION: Vaccination for COVID-19 in the IEI population appears safe and attenuates COVID-19 severity.

Respiratory Comorbidities Associated with Bronchiectasis in Patients with Common Variable Immunodeficiency in the USIDNET Registry.

BACKGROUND: Bronchiectasis is a major respiratory complication in patients with common variable immunodeficiency (CVID) and is associated with recurrent pulmonary infections. However, it is unclear whether other infections or non-infectious respiratory conditions are related to its development. OBJECTIVE: To identify respiratory comorbidities associated with bronchiectasis in patients with CVID. METHODS: A total of 1470 CVID patients enrolled in the USIDNET registry were included in a cross-sectional analysis. The primary outcome of our study was to determine the clinical characteristics and other respiratory conditions associated with respiratory comorbidities and physician-reported bronchiectasis. RESULTS: One hundred ninety-seven CVID patients were noted to have bronchiectasis (13.4%). Affected patients were significantly older than patients without bronchiectasis (median age 54 years vs. 49 years, p = 0.0004). These patients also had lower serum IgA (13 mg/dL IQR 60 mg/dL vs. 28.4 mg/dL IQR 66 mg/dL, p = 0.000). Notably, chronic rhinosinusitis (OR = 1.69 95%CI 1.05-2.75), sinusitis (OR = 2.06 95%CI 1.38-3.09), pneumonia (OR = 2.70 95%CI 1.88-3.88), COPD (OR = 2.66 95%CI 1.51-4.67), and interstitial lung disease (OR = 2.34 95%CI 1.41-3.91) were independently associated with the development of bronchiectasis in this population. CONCLUSION: These data suggest that lower and upper respiratory infections, chronic lower airway disease, and interstitial lung diseases are independently associated with bronchiectasis in CVID patients. Further study into predisposing conditions related to the development of bronchiectasis in CVID patients may allow prediction and early intervention strategies to prevent the development of this complication.

[Chilaiditi’'s sign and syndrome: rare conditions but diagnostically important in pediatrics. Clinical cases]. / Signo y síndrome de Chilaiditi: condiciones infrecuentes pero con importancia diagnóstica en pediatría. Casos clínicos.

Although infrequent, bowel interposition between diaphragm and liver, Chilaiditi’s sign or syndrome (without or with gastrointestinal symptoms), are a major clinical condition given the possibilities of differential diagnosis, such as pneumoperitoneum, diaphragmatic hernia and subphrenic abscess. OBJECTIVE: To report the cases of two preschool patients with Chilaiditi´s sign and syndrome, as well as to highlight the importance of this clinical condition. CLINICAL CASES: Case 1: A male preschooler evaluated by respiratory disease without abdominal symptoms. Thorax X-ray shows left retrocardiac infiltrates and air in right subdiaphragmatic region. Previous radiographies shows the same image. He was diagnosed with Chilaiditi sign associated with pneumonia, antibiotics were used before discharge. Case 2: A female preschooler, evaluated by abdominal distention and constipation. A previous thorax X-ray shows bowel interposition between diaphragm and liver. Barium enema confirmed the findings. Blood test were normal. A Chilaiditi's syndrome was diagnosed. She received medical treatment with favorable evolution. CONCLUSION: These cases highlight the importance of this clinical condition that, despite being infrequent, constitutes a diagnostic challenge in the emergency services.

Endocrine Abnormalities and Growth Characterization in Colombian Pediatric Patients with 22q11 Deletion Syndrome

Objective: Several endocrine manifestations have been described in patients with 22q11 deletion syndrome, including growth retardation, hypoparathyroidism, and thyroid disorders. This study aimed to characterize these abnormalities in a Colombian retrospective cohort of children with this condition. Methods: A retrospective study comprising a cohort of children with 22q11 deletion syndrome in Medellín, Colombia followed up between 2011 and 2017 was conducted. Results: Thirty-seven patients with a confirmed diagnosis of 22q11 deletion syndrome were included. 37.8% had some endocrinopathy, the most frequent being hypoparathyroidism (21.6%), followed by hypothyroidism (13.5%), hyperthyroidism (2.7%) and growth hormone deficiency (2.7%). There was wide heterogeneity in the clinical presentation, with late onset of severe hypocalcemia associated with seizure or precipitated in postoperative cardiac surgery, which highlights the importance of continuous follow-up as indicated by the guidelines. Short stature was mainly related to nutritional factors. Growth monitoring is required with the use of syndrome-specific charts and careful monitoring of the growth rate. Conclusion: As previously reported, a significant proportion of patients with endocrine abnormalities were found in this cohort. This highlights that it is essential to carry out an adequate multidisciplinary follow-up, based on the specific clinical guidelines, in order to avoid serious complications such as convulsions due to hypocalcemia. It is important to track size with curves specific to the syndrome and analyze the growth rate.

Late-Onset Autoimmune Lymphoproliferative Syndrome in a Costa Rican Woman.

Autoimmune lymphoproliferative syndrome (ALPS) is a primary immune regulatory disorder (PIRD). This disease usually develops during childhood. However, atypically, some cases may have their onset in adulthood. We report the case of a 44-year-old woman with a history of autoimmune hemolytic anemia at 33 years old. The patient presented due to asthenia and a large, painful lymph node in the left axillary region for the last four months. Enlargement of the axillary and inguinal lymph nodes was found by mammography, breast, and abdominal ultrasounds. An excisional biopsy of the axillary lymph node conglomerate did not document immunophenotypical alterations of T or B lymphocytes but showed progressive transformation of germinal centers with reactive follicular hyperplasia. The lymph node cytometry did not show a malignant phenotype. The immunological work-up documented IgG and IgA hypergammaglobulinemia and slightly decreased IgM; the B cell immunophenotype documented a slight increase in CD21low B cells and decreased memory B cells. The blood count was normal. The T cell compartment evidenced 27% CD3+/αß+/γδ-/CD4-/CD8- of the total T CD3+ cells and 15% of the total lymphocytes. A pathogenic heterozygous variant in the FAS gene, exon 9, c.785T>A (p.Ile262Asn), was documented. This variant has not been previously described. This case highlights the importance of considering the diagnosis of ALPS even in adulthood. Genetic conditions such as incomplete penetrance or variable expressivity that depend on factors that are not entirely clear in ALPS, such as epigenetics and environmental factors, among others, could generate the onset of this disease in adulthood in a smaller number of patients.

Susceptibility to thiopurine toxicity by TPMT and NUDT15 variants in Colombian children with acute lymphoblastic leukemia.

Objective: This study aimed to correlate the genetic profile of the NUDT15 and TPMT genes with the side effects of the treatment of pediatric patients with acute lymphoid leukemia who were undergoing maintenance therapy at a tertiary care hospital in 2017. Methods: This was an analytical, longitudinal, observational study in which the genotypes of the genes of interest were determined by PCR allelic discrimination with TaqMan® probes in patients receiving chemotherapy during the maintenance phase in the Pediatric Hematology and Oncology Unit in 2017. Sociodemographic and clinical data corresponding to the first six months of their maintenance chemotherapy were collected, and the correlation between the genotypes obtained and the development of side effects during the maintenance phase of chemotherapy in these patients was evaluated. Results: Seventy pediatric patients were included in the study. Genetic analyses were carried out of these for NUDT15 and TPMT (rs1800462 and rs1800460) on 68 patients, while for the rs1142345 polymorphism, typing was achieved in 42 patients. 4/68 patients were heterozygous for NUDT15, and the same number of patients were heterozygous for rs1800462 and rs1142345, while for rs1800460, 6 heterozygous patients were identified. No statistically significant association was identified between the genetic variants and the outcomes of interest. Conclusion: Studies with a larger population size are needed and the evaluation of other genetic variants that may influence the development of side effects during maintenance chemotherapy.

Objetivo: la finalidad de este estudio fue evaluar las asociaciones entre los perfiles de los genes NUDT15 y TPMT con los efectos adversos del tratamiento de mantenimiento en pacientes pediátricos con Leucemia Linfoblástica Aguda atendidos en un hospital de referencia durante el 2017. Métodos: Este fue un estudio observacional analítico, de corte longitudinal en el que los genotipos de los genes de interés fueron determinados mediante PCR de discriminación alélica con sondas TaqMan® en pacientes que estaban recibiendo quimioterapia de mantenimiento en la Unidad de Oncohematología Pediátrica durante el 2017. Los datos clínicos y sociodemográficos correspondientes a los primeros 6 meses de sus tratamientos de mantenimiento fueron colectados, y se evaluó la correlación entre los genotipos identificados y el desarrollo de efectos secundarios en estos pacientes. Resultados: setenta pacientes fueron incluidos en el estudio, de estos, los análisis genéticos para NUDT15 y TPMT (rs1800462 and rs1800460) fueron realizados en 68 pacientes, en tanto que para el polimorfismo rs1142345 se logró la tipificación en 42 pacientes. 4/68 pacientes fueron heterocigotos para NUDT15 y el mismo número de pacientes fueron heterocigotos para rs1800462 and rs1142345, mientras que para rs1800460, 6 pacientes heterocigotos fueron identificados. No se identificaron asociaciones estadísticamente significantes entre las variants genéticas y los resultados clínicos de interés. Conclusiones: Estos hallazgos resaltan la importancia de realizar estudios de este tipo con un mayor número de sujetos de estudio, así como plantean la necesidad de evaluar otras variantes genéticas que podrían tener algún impacto en el desarrollo de efectos secundarios durante la quimioterapia de mantenimiento.

Pulmonary nodule in pediatrics: Beyond the infection. / Nódulo pulmonar en pediatría: Más allá de la infección.

INTRODUCTION: The study of a pulmonary nodule in pediatrics is a diagnostic challenge where multiple pathologies must be taken into account, especially infections. In developing countries, where tuberculosis infec tion is endemic, it is one of the most likely diagnoses; however, the diagnostic possibility of malig nancy should never be overlooked. OBJECTIVE: To describe a case report of a patient with a pulmonary nodule, that after ruling out the most frequent causes, a primary malignant tumor was diagnosed. CLINICAL CASE: 17-year-old female patient with a one-month history of cough, dyspnea, and hemop tysis, without other symptoms. Since she did not respond to conventional antibiotic management, a chest CT scan with contrast was performed which showed a pulmonary nodule with irregular con tours, and with the bronchoalveolar lavage pulmonary infections were ruled out (pulmonary tuber culosis, fungal infection, and others bacteria). Biopsy of the lesion was performed to complete the study which histopathology was compatible with a mucoepidermoid carcinoma (MEC). The patient underwent right low lobectomy and lymph node resection with good clinical response during three years of follow-up. CONCLUSION: Pulmonary nodule in pediatrics is a finding usually associated with infection, nevertheless, neoplastic conditions have to be considered, not only metastasis but also pri mary pulmonary malignant lesions due to prognosis implications.

Consenso colombiano de la enfermedad inflamatoria intestinal pediátrica

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Introduction: Pediatric ulcerative colitis (CUP), pediatric Crohn's disease (PCD), and pediatric inflammatory bowel disease not classifiable (PIDNCID) have clinical and psychosocial particularities that differentiate them from those of adults and may condition different therapeutic approaches due to possible nutritional, growth and developmental repercussions, representing a challenge for the pediatrician and gastroenterologist. Objective: Develop expert consensus evidence-based recommendations for the timely and safe diagnosis and treatment of Pediatric Inflammatory Bowel Disease (PID) in children under 18 years of age for professionals caring for these patients and healthcare payers. Methodology: Through a panel of experts from the Colombian College of Pediatric Gastroenterology, Hepatology and Nutrition (COLGAHNP) and a multidisciplinary group, 35 questions were asked regarding the clinical picture, diagnosis, and treatment of PID. Through a critical review and analysis of the literature with particular emphasis on the main clinical practice guidelines (CPGs), randomized clinical trials (RCTs), and meta-analyses of the last ten years, from which the experts made 77 recommendations that responded to each of the research questions with their respective practical points. Subsequently, each of the statements was voted on within the developer group, including the statements that achieved > 80%. Results: All statements scored > 80%. PID has greater extension, severity, and evolution towards stenosis, perianal disease, extraintestinal manifestations, and growth retardation compared to adult patients, so its management should be performed by multidisciplinary groups led by pediatric gastroenterologists and prepare them for a transition to adulthood. Porto's criteria allow a practical classification of PID. In CPE, we should use the Paris classification and perform ileocolonoscopy and esophagogastroduodenoscopy, since 50% have upper involvement, using the SES-CD (UCEIS/Mayo in CUP) and taking multiple biopsies. Initial labs should include inflammatory markers and fecal calprotectin and rule out intestinal infections. Treatment, induction, and maintenance of PID should be individualized and decided according to risk stratification. Follow-up should use PCDAI and PUCAI for the last 48 hours. Immunologists and geneticists should evaluate patients with early and infantile PID. Conclusion: A consensus guideline is provided with evidence-based recommendations on timely and safe diagnosis and treatments in patients with ILD.

High frequency of Panton-Valentine leukocidin in Staphylococcus aureus causing pediatric infections in the city of Cartagena-Colombia.

Panton-Valentine leucocidin (PVL) is a pore-forming toxin that has been epidemiologically associated with CA-MRSA infections. However, its role in the pathogenicity of Staphylococcus aureus is still unclear. We evaluated the prevalence of PVL-coding genes in methicillin-resistant (MRSA) and methicillin-sensitive (MSSA) isolates that cause infections in pediatric patients in the city of Cartagena, Colombia. We obtained S. aureus isolates from patients at the Napoleon Franco Pareja Children's Hospital in Cartagena. Then, we evaluated the presence of the nuc, mecA, and PVL genes in these isolates by multiplex PCR and determined the antibiotic susceptibility profiles using CLSI standards. We further correlated methicillin susceptibility and the presence of PVL genes with clinical variables. Overall PVL prevalence in S. aureus isolates was 73.91%, with a frequency of 80.92% among MRSA isolates and 67.59% among MSSA. We found a correlation between erythromycin resistance and lack of PVL and found that PVL+ cases were more common in older patients. We found a high PVL prevalence in both MRSA and MSSA isolates, in concordance with previous regional reports.

Susceptibility to thiopurine toxicity by TPMT and NUDT15 variants in Colombian children with acute lymphoblastic leukemia / Asociación de variantes genéticas en TPMT y NUDT15 con los eventos de toxicidad durante la terapia de mantenimiento en niños colombianos con Leucemia Linfoide Aguda

Abstract Objective: This study aimed to correlate the genetic profile of the NUDT15 and TPMT genes with the side effects of the treatment of pediatric patients with acute lymphoid leukemia who were undergoing maintenance therapy at a tertiary care hospital in 2017. Methods: This was an analytical, longitudinal, observational study in which the genotypes of the genes of interest were determined by PCR allelic discrimination with TaqMan® probes in patients receiving chemotherapy during the maintenance phase in the Pediatric Hematology and Oncology Unit in 2017. Sociodemographic and clinical data corresponding to the first six months of their maintenance chemotherapy were collected, and the correlation between the genotypes obtained and the development of side effects during the maintenance phase of chemotherapy in these patients was evaluated. Results: Seventy pediatric patients were included in the study. Genetic analyses were carried out of these for NUDT15 and TPMT (rs1800462 and rs1800460) on 68 patients, while for the rs1142345 polymorphism, typing was achieved in 42 patients. 4/68 patients were heterozygous for NUDT15, and the same number of patients were heterozygous for rs1800462 and rs1142345, while for rs1800460, 6 heterozygous patients were identified. No statistically significant association was identified between the genetic variants and the outcomes of interest. Conclusion: Studies with a larger population size are needed and the evaluation of other genetic variants that may influence the development of side effects during maintenance chemotherapy.

Resumen Objetivo: la finalidad de este estudio fue evaluar las asociaciones entre los perfiles de los genes NUDT15 y TPMT con los efectos adversos del tratamiento de mantenimiento en pacientes pediátricos con Leucemia Linfoblástica Aguda atendidos en un hospital de referencia durante el 2017. Métodos: Este fue un estudio observacional analítico, de corte longitudinal en el que los genotipos de los genes de interés fueron determinados mediante PCR de discriminación alélica con sondas TaqMan® en pacientes que estaban recibiendo quimioterapia de mantenimiento en la Unidad de Oncohematología Pediátrica durante el 2017. Los datos clínicos y sociodemográficos correspondientes a los primeros 6 meses de sus tratamientos de mantenimiento fueron colectados, y se evaluó la correlación entre los genotipos identificados y el desarrollo de efectos secundarios en estos pacientes. Resultados: setenta pacientes fueron incluidos en el estudio, de estos, los análisis genéticos para NUDT15 y TPMT (rs1800462 and rs1800460) fueron realizados en 68 pacientes, en tanto que para el polimorfismo rs1142345 se logró la tipificación en 42 pacientes. 4/68 pacientes fueron heterocigotos para NUDT15 y el mismo número de pacientes fueron heterocigotos para rs1800462 and rs1142345, mientras que para rs1800460, 6 pacientes heterocigotos fueron identificados. No se identificaron asociaciones estadísticamente significantes entre las variants genéticas y los resultados clínicos de interés. Conclusiones: Estos hallazgos resaltan la importancia de realizar estudios de este tipo con un mayor número de sujetos de estudio, así como plantean la necesidad de evaluar otras variantes genéticas que podrían tener algún impacto en el desarrollo de efectos secundarios durante la quimioterapia de mantenimiento.

Portación de Candida spp. en cavidad oral en diabéticos y no diabéticos / Carrying of Candida spp. in the oral cavity in diabetic and non-diabetic patients

Introducción: Candida spp. es un habitante normal de la microbiota humana, que puede originar infecciones superficiales y sistémicas de carácter oportunista. En pacientes diabéticos se incrementa el riesgo de infecciones por esta levadura, lo cual estaría determinado por la portación de Candida spp. Esta portación es variable, así se observa en cavidad oral desde 13,7 al 64 por ciento. Objetivo: establecer los porcentajes de colonización y posibles factores asociados en este grupo de alto riesgo. Método: se realizó un estudio descriptivo en un total de 172 pacientes diabéticos y no diabéticos. Las muestras de enjuague bucal se sembraron en agar Sabouraud y CHROMagar Candida. Los aislamientos se sometieron a pruebas fenotípicas y a reacción en cadena de la polimerasa múltiple para su identificación. Las variables demográficas, los hábitos de higiene oral, el uso de prótesis dental, así como los niveles de hemoglobina glucosilada se evaluaron para determinación de frecuencias y asociación por chi2 y análisis multivariado, mediante el programa SPSS versión 19.0. Resultados: el porcentaje de colonización en el total de la población diabética y no diabética (n= 172) fue de 33,7 por ciento. La distribución por especies fue de Candida albicans (63,8 por ciento), Candida glabrata (10,3 por ciento), Candida tropicalis (6,9 por ciento), Candida krusei (5,2 por ciento), Candida dubliniensis (3,4 por ciento), Candida parapsilosis (3,4 por ciento), Candida lusitaniae (1,7 por ciento), Candida guilliermondii (1,7 por ciento) y Candida spp. (no identificada, 3,4 por ciento). En sujetos no diabéticos el porcentaje de colonización fue de 27,9 por ciento y en diabéticos de 36,9 por ciento. En los sujetos del estudio se encontró que 14,9 por ciento tenía control glúcemico por los niveles de hemoglobina glucosilada, el 57,6 por ciento utilizaba prótesis dentales y el 63,9 por ciento practicaba higiene oral regular. Conclusión: Candida albicans es la especie predominante en ambos grupos, con un porcentaje significativo de las especies no albicans en estos pacientes. El uso de prótesis dental es un factor coadyuvante para la colonización por especies del género Candida(AU)

Introduction: Candida spp. is a normal inhabitant of the human microbiota, which can cause superficial and systemic infections of an opportunistic nature. In diabetic patients the risk of infections by this yeast increases, which would be determined by the carrying of Candida spp. This carrying is variable, as observed in the oral cavity from 13.7 to 64 percent. Objective: to establish the percentages of colonization and possible associated factors in this high-risk group. Method: a descriptive study was carried out in a total of 172 diabetic and non-diabetic patients. Mouthwash samples were seeded on Sabouraud agar and CHROMagar Candida. The isolates were subjected to phenotypic tests and to a multiple polymerase´s chain reaction for identification. Demographic variables, oral hygiene habits, the use of dental prostheses, as well as glycosylated hemoglobin levels were evaluated for frequency and association determination by chi2 and multivariate analysis, using the SPSS program version 19.0. Results: the percentage of colonization in the total of the diabetic and non-diabetic population (n= 172) was 33.7 percent. The distribution by species was Candida albicans (63.8 percent), Candida glabrata (10.3 percent), Candida tropicalis (6.9 percent), Candida krusei (5.2 percent), Candida dubliniensis (3.4 percent), Candida parapsilosis (3.4 percent), Candida lusitaniae (1.7 percent), Candida guilliermondii (1.7 percent), and Candida spp. (unidentified, 3.4 percent). In non-diabetic patients the percentage of colonization was 27.9 percent and in diabetics 36.9 percent. In the study´s patients, it was found that 14.9 percent had glycemic control by glycosylated hemoglobin levels, 57.6 percent used dental prostheses, and 63.9 percent practiced regular oral hygiene. Conclusion: Candida albicans was the predominant specie in both groups, with a significant percentage of the non-albicans species in these patients. The use of dental prostheses was a contributory factor for colonization by species of the genus Candida(AU)

Signo y síndrome de Chilaiditi: condiciones infrecuentes pero con importancia diagnóstica en pediatría: casos clínicos / Chilaiditi’s sign and syndrome: rare conditions but diagnostically important in pediatrics: clinical cases

Aunque poco frecuente, la interposición de intestino entre el diafragma y el hígado, signo o síndrome de Chilaiditi (en relación a la ausencia o presencia de síntomas gastrointestinales), constituye una condición clínica de importancia dadas las posibilidades de diagnóstico diferencial, tales como: neumoperitoneo, hernia diafragmática y absceso subfrénico. OBJETIVO: Presentar dos preescolares con signo y síndrome de Chilaiditi, así como resaltar la importancia de esta condición clínica. CASOS CLÍNICOS: Caso 1: Preescolar varón evaluado por cuadro respiratorio sin síntomas abdominales. La radiografía de tórax mostró infiltrados retrocardíacos izquierdos y presencia de aire en región subdiafragmática derecha. En radiografías previas se encontraba la misma imagen. Se concluyó que se trataba de un signo de Chilaiditi asociado a un cuadro de neumonía, se trató con antibioterapia y se dio de alta. Caso 2: Preescolar de sexo femenino, evaluada por distensión abdominal y constipación. Radiografía de tórax, solicitada previamente por cuadro respiratorio, con imagen de colon derecho situado entre el hemidiafragma y el hígado. Enema baritado de colon confirmó los hallazgos. Exámenes de laboratorio normales. Se concluyó síndrome de Chilaiditi, se indicó tratamiento médico, con buena evolución. CONCLUSIÓN: Se resalta la importancia de esta condición clínica que pese a ser infrecuente constituye un reto diagnóstico en los servicios de urgencia.

Although infrequent, bowel interposition between diaphragm and liver, Chilaiditi’s sign or syndrome (without or with gastrointestinal symptoms), are a major clinical condition given the possibilities of differential diagnosis, such as pneumoperitoneum, diaphragmatic hernia and subphrenic abscess. Objective: To report the cases of two preschool patients with Chilaiditi´s sign and syndrome, as well as to highlight the importance of this clinical condition. Clinical cases: Case 1: A male preschooler evaluated by respiratory disease without abdominal symptoms. Thorax X-ray shows left retrocardiac infiltrates and air in right subdiaphragmatic region. Previous radiographies shows the same image. He was diagnosed with Chilaiditi sign associated with pneumonia, antibiotics were used before discharge. Case 2: A female preschooler, evaluated by abdominal distention and constipation. A previous thorax X-ray shows bowel interposition between diaphragm and liver. Barium enema confirmed the findings. Blood test were normal. A Chilaiditi's syndrome was diagnosed. She received medical treatment with favorable evolution. Conclusion: These cases highlight the importance of this clinical condition that, despite being infrequent, constitutes a diagnostic challenge in the emergency services.