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BACKGROUND: Despite the substantial burden caused by childhood cancer globally, childhood cancer incidence obtained in a nationwide childhood cancer registry and the accessibility of relevant health services are still unknown in China. We comprehensively assessed the most up-to-date cancer incidence in Chinese children and adolescents, nationally, regionally, and in specific population subgroups, and also examined the association between cancer incidence and socioeconomic inequality in access to health services. METHODS: In this national cross-sectional study, we used data from the National Center for Pediatric Cancer Surveillance, the nationwide Hospital Quality Monitoring System, and public databases to cover 31 provinces, autonomous regions, and municipalities in mainland China. We estimated the incidence of cancer among children (aged 0-14 years) and adolescents (aged 15-19 years) in China through stratified proportional estimation. We classified regions by socioeconomic status using the human development index (HDI). Incidence rates of 12 main groups, 47 subgroups, and 81 subtypes of cancer were reported and compared by sex, age, and socioeconomic status, according to the third edition of the International Classification of Childhood Cancer. We also quantified the geographical and population density of paediatric oncologists, pathology workforce, diagnoses and treatment institutions of paediatric cancer, and paediatric beds. We used the Gini coefficient to assess equality in access to these four health service indicators. We also calculated the proportions of cross-regional patients among new cases in our surveillance system. FINDINGS: We estimated the incidence of cancer among children (aged 0-14 years) and adolescents (aged 15-19 years) in China from Jan 1, 2018, to Dec 31, 2020. An estimated 121â145 cancer cases were diagnosed among children and adolescents in China between 2018 and 2020, with world standard age-standardised incidence rates of 122·86 (95% CI 121·70-124·02) per million for children and 137·64 (136·08-139·20) per million for adolescents. Boys had a higher incidence rate of childhood cancer (133·18 for boys vs 111·21 for girls per million) but a lower incidence of adolescent cancer (133·92 for boys vs 141·79 for girls per million) than girls. Leukaemias (42·33 per million) were the most common cancer group in children, whereas malignant epithelial tumours and melanomas (30·39 per million) surpassed leukaemias (30·08 per million) in adolescents as the cancer with the highest incidence. The overall incidence rates ranged from 101·60 (100·67-102·51) per million in very low HDI regions to 138·21 (137·14-139·29) per million in high HDI regions, indicating a significant positive association between the incidence of childhood and adolescent cancer and regional socioeconomic status (p<0·0001). The incidence in girls showed larger variation (48·45% from the lowest to the highest) than boys (36·71% from lowest to highest) in different socioeconomic regions. The population and geographical densities of most health services also showed a significant positive correlation with HDI levels. In particular, the geographical density distribution (Gini coefficients of 0·32-0·47) had higher inequalities than population density distribution (Gini coefficients of 0·05-0·19). The overall proportion of cross-regional patients of childhood and adolescent cancer was 22·16%, and the highest proportion occurred in retinoblastoma (56·54%) and in low HDI regions (35·14%). INTERPRETATION: Our study showed that the burden of cancer in children and adolescents in China is much higher than previously nationally reported from 2000 to 2015. The distribution of the accessibility of health services, as a social determinant of health, might have a notable role in the socioeconomic inequalities in cancer incidence among Chinese children and adolescents. With regards to achieving the Sustainable Development Goals, policy approaches should prioritise increasing the accessibility of health services for early diagnosis to improve outcomes and subsequently reduce disease burdens, as well as narrowing the socioeconomic inequalities of childhood and adolescent cancer. FUNDING: National Major Science and Technology Projects of China, National Natural Science Foundation of China, Chinese Academy of Engineering Consulting Research Project, Wu Jieping Medical Foundation, Beijing Municipal Administration of Hospitals Incubating Program.
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Leucemia , Neoplasias , Adolescente , Niño , China/epidemiología , Estudios Transversales , Femenino , Servicios de Salud , Accesibilidad a los Servicios de Salud , Humanos , Incidencia , Masculino , Neoplasias/diagnóstico , Neoplasias/epidemiología , Factores SocioeconómicosRESUMEN
OBJECTIVE: To evaluate the efficacy and safety of microwave ablation (MWA) for the treatment of symptomatic benign thyroid nodules in children. METHODS: A retrospective study of MWA for the treatment of 34 symptomatic benign thyroid nodules in 25 children was conducted. Volume reduction ratio (VRR), technique efficacy, symptom score, cosmetic score, and thyroid function were used to evaluate the efficacy of the technique. The associated complications and side effects were recorded. RESULTS: The participants were followed for at least 6 months (median 12 months, range 6-48 months). After MWA treatment, the volumes of the targeted nodules decreased gradually (median volume 5.86 mL before MWA and 0.34 mL at the final follow-up assessment), the VRR achieved was up to 85.03% at the final follow-up assessment, and the technical efficacy at this time was 91.2%. The subjective and objective nodule-related symptoms were also ameliorated. The circulating hormone concentrations reflecting thyroid function remained within their normal ranges in all the participants after one month of follow-up. The procedure had no major complications. CONCLUSIONS: MWA seems to be an effective and safe technique for the treatment of symptomatic benign thyroid nodules in pediatric patients. CLINICAL RELEVANCE STATEMENT: Microwave ablation is a safe and effective method to treat symptomatic benign thyroid nodules in pediatric patients. This treatment may be selected if the patient or parents are not suitable or refuse to undergo surgery. KEY POINTS: ⢠Microwave ablation is effective in reducing the volume of benign thyroid nodules and ameliorating nodule-related symptoms in pediatric patients. ⢠Microwave ablation is a safe method in children, with low complications. ⢠Microwave ablation does not affect the circulating thyroid hormone concentrations of children.
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Wound healing is a common occurrence. However, delayed healing and aberrant scarring result in pathological wound healing. Accordingly, a scarless wound healing remains a significant clinical challenge. In this study, we constructed hyaluronic acid (HA)-modified and verteporfin (VP)-loaded polylactic acid (PLA) nanogels (HA/VP-PLA) to promote scarless wound healing by accelerating wound re-epithelialization and controlling scar formation. Owing to the unique structure of HA incorporating and coating in VP-loaded PLA nanoparticles, HA/VP-PLA could be topically applied on wound to achieve targeted delivery to fibroblasts. Then, HA/VP-PLA released HA and lactic acid (LA) to stimulate the proliferation and migration of fibroblasts, as well as VP to inhibit Yes-associated protein (YAP) expression and nuclear localization to suppress fibrosis. In vitro (skin fibroblasts) and in vivo (rat and rabbit models) experiments strongly suggested that HA/VP-PLA promoted scarless wound healing by accelerating wound re-epithelialization and controlling scar formation. Therefore, our work provides a feasible strategy for scarless wound healing, and the sophisticated HA/VP-PLA exhibit a great potential for clinical applications.
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Cicatriz , Ácido Hialurónico , Ratas , Animales , Conejos , Cicatriz/tratamiento farmacológico , Cicatriz/prevención & control , Cicatriz/metabolismo , Ácido Hialurónico/química , Repitelización , Nanogeles , Verteporfina , Cicatrización de Heridas , Poliésteres , Piel/metabolismoRESUMEN
BACKGROUND: There are a limited number of validated questionnaires available for use in the clinical screening for allergic rhinitis (AR) in children ≤3 years old. We developed a novel self-reported questionnaire and assessed its accuracy and reliability. METHODS: After establishing a pool of items, which were screened by experts, the Young Children Allergic Rhinitis Questionnaire (YCAR-Q) was administered to a birth cohort in the Shunyi District (Beijing, China). The electronic version of the YCAR-Q was distributed through the online community. Children were invited to visit a physician for examination. The diagnostic criteria included symptoms, physical examination findings, and specific serum immunoglobulin E tests. Each item on the questionnaire was evaluated, and the questionnaire's internal consistency, content validity, criterion-related validity, and diagnostic accuracy were assessed. RESULTS: The six-item YCAR-Q was distributed to 7423 parents, and 3037 valid questionnaires were recovered. In total, 1521 children visited a physician for examination, of which 82 were found to have AR. In terms of internal consistency, Cronbach's coefficient was 0.777 and all six questionnaire items were retained. The average scale-level content validity index value was 1. The area under the curve was 0.759. The total scores ranged from 0 to 6, and the cutoff value for diagnosing AR was 3, with a sensitivity of 68.29% and a specificity of 76.58%. CONCLUSIONS: This cross-sectional study indicated that the YCAR-Q could detect AR in children ≤3 years old. This brief and simple test may be used effectively in clinical practice.
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Rinitis Alérgica , Niño , Preescolar , Estudios Transversales , Humanos , Tamizaje Masivo , Reproducibilidad de los Resultados , Rinitis Alérgica/diagnóstico , Rinitis Alérgica/epidemiología , Encuestas y CuestionariosRESUMEN
BACKGROUND: The incidence of thyroid cancer is fast increasing in both adults and children. The pediatric thyroid cancer had often already progressed to a more advanced stage of the disease at diagnosis. Early detection of pediatric thyroid cancer has been a problem for many years. Lipocalin-2 (Lcn2) has been reported to be over-expressed in cancers of diverse histological origin and it facilitates tumorigenesis by promoting survival, growth, and metastasis. METHODS: The plasma Lcn2 concentration of 28 Chinese papillary thyroid cancer (PTC) children and 24 healthy controls was measured. Immunostaining for Ki-67 of tumor tissue from PTC children was performed. The expression levels of Lcn2 and NFκB in PTC tissue and peri-carcinoma tissue of PTC children were measured through Western blot. RESULTS: The plasma concentration of Lcn2 was significantly elevated in pediatric PTC patients compared with healthy controls. Besides, the plasma Lcn2 concentration significantly correlated with clinical characteristics, NFκB level, and Ki-67 positive rate of nucleus in tissue of PTC. CONCLUSION: This is the first study to evaluate the plasma Lcn2 in pediatric PTC patients. It is possible that the plasma Lcn2 may be a new biomarker of pediatric thyroid cancer. Further studies are needed to explore the definite role and mechanism of Lcn2 in thyroid cancer, which will help to explore novel diagnostic or therapeutic strategies.
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Carcinoma/metabolismo , Carcinoma/patología , Lipocalina 2/sangre , Neoplasias de la Tiroides/metabolismo , Neoplasias de la Tiroides/patología , Adolescente , Estudios de Casos y Controles , Niño , Femenino , Humanos , Masculino , FN-kappa B/metabolismo , Activación Transcripcional , Regulación hacia ArribaRESUMEN
Osmoreception is essential for systemic osmoregulation, a process to stabilize the tonicity and volume of the extracellular fluid through regulating the ingestive behaviour, sympathetic outflow and renal function. The sensation of osmotic changes by osmoreceptor neurons is mediated by ion channels that detect the change of osmolarity in extracellular fluid. However, the molecular identity of these channels remains mysterious. AtCSC1and OSCA1,two closely related paralogues from Arabidopsis, have been demonstrated to form hyperosmolarity activated ion channels, which makes their mammalian orthologues-the members of TMEM63 proteins, possible candidates for osmoreceptor transduction channel. To test this possibility, we cloned the cDNAs of all the three members of the mouse TMEM63 family, TMEM63A, TMEM63B and TMEM63C from the mRNA from mouse brain. When all of the three subtypes of TMEM63 proteins were co-expressed in HEK293 cells, we recorded membrane currents evoked by hypertonic stimulation in these cells. However, the cells expressing the combinations of any two subtypes of TMEM63 proteins could not exhibit any hyperosmolarity evoked currents. Thus, all the three members of TMEM63 proteins are required to constitute a hyperosmolarity activated ion channel. We propose that the TMEM63 proteins may serve as an osmolarity sensitive ion channel for the osmoreception. Copyright © 2016 John Wiley & Sons, Ltd.
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Activación del Canal Iónico , Canales Iónicos/metabolismo , Proteínas de la Membrana/metabolismo , Animales , Células HEK293 , Humanos , Soluciones Hipertónicas/farmacología , Activación del Canal Iónico/efectos de los fármacos , Ratones , Concentración Osmolar , FilogeniaRESUMEN
OBJECTIVE: To evaluate the relationship and strength of association for alcohol drinking and tea consumption with the riskS of nasopharynx cancer among Chinese population so as to provide control rationales for nasopharynx cancer in China. METHODS: A systematic search of 3 Chinese electronic databases (CNKI, VIP, Wanfang) and 3 English databases (Pubmed, ScienceDirect and SpringerLink) up to March 2013 was performed. Two reviewers independently conducted the literature search, examined eligibility and performed data extraction and quality evaluations. Pooled odd ratio (OR) value and 95%CI value were calculated with random-effects model weighted with inverse of variances. RESULTS: A total of 14 studies (including 3 cohort and 11 case-control) involving 6559 cases of nasopharynx cancer and 10 567 controls from 6 provinces were included. The pooled OR between alcohol drinking and risks of nasopharynx cancer was 1.12 (95%CI: 0.98-1.26; I(2) = 44.5%, P = 0.037). Compared with the non-drinkers, the risks of nasopharynx cancer for regular drinkers and occasional drinkers were 1.18 (95%CI: 1.00-1.38; I(2) = 0.0%, P = 0.578) and 0.76 (95%CI: 0.65-0.89; I(2) = 33.4%, P = 0.212). And the association of tea consumption with the risks of nasopharynx cancer was 0.53 (95%CI: 0.43-0.60; I(2) = 17.9%, P = 0.301). CONCLUSIONS: In China, occasional alcohol drinking may decrease the risks of nasopharynx cancer while regular drinking elevates the risks. And there is significantly protective effect for tea consumption on the risks of nasopharynx cancer.
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Consumo de Bebidas Alcohólicas/epidemiología , Conducta Alimentaria , Neoplasias Nasofaríngeas/epidemiología , Té , China/epidemiología , Humanos , Factores de RiesgoRESUMEN
OBJECTIVES: To explore the differences in the efficacy and safety of oral sirolimus and sildenafil in the treatment of pediatric intractable lymphatic malformations (LMs). METHODS: From January 2014 to May 2022, we retrospectively enrolled children with intractable LMs treated with oral drugs (sirolimus or sildenafil) and divided the patients into sirolimus and sildenafil groups from Beijing Children's Hospital (BCH). Clinical features, treatment, and follow-up data were collected and analyzed. The indicators were the ratio of reduction in lesion volume pre and posttreatment, the number of patients with improved clinical symptoms, and adverse reactions to the two drugs. RESULTS: Twenty-four children in the sildenafil group and 31 children in the sirolimus group were included in the present study. The effective rate in the sildenafil group was 54.2% (13/24), with a median lesion volume reduction ratio of 0.32 (-0.23, 0.89) and clinical symptoms improved in 19 patients (79.2%). On the contrary, the effective rate in the sirolimus group was 93.5% (29/31), with a median lesion volume reduction ratio of 0.68 (0.34, 0.96), and clinical symptoms improved in 30 patients (96.8%). There were significant differences (p < 0.05) between the two groups. Regarding safety, four patients in the sildenafil group and 23 patients in the sirolimus group with mild adverse reactions were reported. CONCLUSION: Both sildenafil and sirolimus can reduce the volume of LMs and improve clinical symptoms in partial patients with intractable LMs. Sirolimus is more effective than sildenafil and the adverse reactions associated with both drugs are mild and controllable. LEVEL OF EVIDENCE: III Laryngoscope, 133:3192-3199, 2023.
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Anomalías Linfáticas , Malformaciones Vasculares , Niño , Humanos , Citrato de Sildenafil/efectos adversos , Sirolimus/efectos adversos , Estudios Retrospectivos , Resultado del Tratamiento , Anomalías Linfáticas/tratamiento farmacológicoRESUMEN
Purpose The cervicothoracic junction (CTJ) lesions in children is rare. Surgical treatment for lesions at the cervicothoracic junction is challenging due to the presence of the great vessels and other thoracic structures. There are no criteria that help select a surgical approach to manage cervicothoracic lesions in children so far. This study focuses on the cervicothoracic junction lesions in children(C7-T4) and provides experience for the appropriate surgical approach for them.Methods: This retrospective study enrolled 18 children with cervicothoracic junction lesions who underwent surgical treatment in our Hospital from January 2015 to September 2019. They were evaluated with preoperative CT or MR imaging and diagnosed postoperatively by pathological examination.Results: This study included 2 patients with congenital lesions, 4 patients with benign lesions, and 12 patients with malignant lesions. Lesions with a margin below C7-T3, including benign and malignant tumors could be resected using a simple low anterior cervical approach (LACA). Congenital lesions and benign lesions with a margin below T4 could also be treated with this approach. Two-thirds of the malignant lesions below T4 were resected through the LACA combined with video-assisted thoracoscopic surgery (VATS). 1 patient with malignant lesion extending to T4 was removed by the LACA combined with posterolateral thoracotomy.Conclusions: The lesions at the cervicothoracic junction (C7-T4) in children may be managed with the simple LACA used in most patients. For malignancies extending to the T4 level, LACA and VATS could be performed in combination to resect lesions completely and invasively.
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Vértebras Cervicales , Vértebras Torácicas , Vértebras Cervicales/diagnóstico por imagen , Vértebras Cervicales/cirugía , Niño , Humanos , Imagen por Resonancia Magnética , Estudios Retrospectivos , Vértebras Torácicas/diagnóstico por imagen , Vértebras Torácicas/cirugíaRESUMEN
Different newborn screening (NBS) programs have been practiced in many countries since the 1960s. It is of considerable interest whether next-generation sequencing is applicable in NBS. We have developed a panel of 465 causative genes for 596 early-onset, relatively high incidence, and potentially actionable severe inherited diseases in our Newborn Screening with Targeted Sequencing (NESTS) program to screen 11,484 babies in 8 Women and Children's hospitals nationwide in China retrospectively. The positive rate from preliminary screening of NESTS was 7.85% (902/11,484). With 45.89% (414/902) follow-up of preliminary positive cases, the overall clinically confirmative diagnosis rate of monogenic disorders was 12.07% (50/414), estimating an average of 0.95% (7.85% × 12.07%) clinical diagnosis rate, suggesting that monogenic disorders account for a considerable proportion of birth defects. The disease/gene spectrum varied in different regions of China. NESTS was implemented in a hospital by screening 3923 newborns to evaluate its clinical application. The turn-around time of a primary report, including the sequencing period of < 7 days, was within 11 days by our automatic interpretation pipeline. Our results suggest that NESTS is feasible and cost-effective as a first-tier NBS program, which will change the status of current clinical practice of NBS in China.
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Secuenciación de Nucleótidos de Alto Rendimiento , Tamizaje Neonatal , Niño , China/epidemiología , Femenino , Secuenciación de Nucleótidos de Alto Rendimiento/métodos , Humanos , Lactante , Recién Nacido , Masculino , Tamizaje Neonatal/métodos , Estudios RetrospectivosRESUMEN
BACKGROUND: Thyroid carcinoma is a common pediatric head and neck cancer, of which papillary thyroid cancer (PTC) is the most common type. Previously, we found that thyroid peroxidase (TPO) and aldehyde oxidase 1 (AOX1) were differentially expressed in PTC. This study explored the clinical importance of TPO and AOX1 in the diagnosis and prognosis of PTC in children. METHODS: Both TPO and AOX1 expression in PTC were analyzed using datasets from Gene Expression Omnibus (GEO). TPO and AOX1 protein levels in plasma from patients with PTC and non-tumor controls were detected via enzyme-linked immunosorbent assay (ELISA). The diagnostic accuracy of TPO and AOX1 was assessed using receiver operating characteristic (ROC) curve analysis. The association between gene expression levels and patient survival was explored using the Kaplan-Meier plotter online database. RESULTS: The results revealed that TPO and AOX1 expression was significantly downregulated in four independent datasets (GSE33630, GSE27155, GSE3678, and GSE3467). TPO and AOX1 protein levels in blood plasma were significantly decreased in patients with PTC. Quantitative analysis demonstrated that TPO and AOX1 levels in plasma had satisfactory predictive performance and the ability to discriminate PTC from healthy samples. Prognostic analysis demonstrated that low levels of TPO and AOX1 were markedly associated with poor survival in patients with PTC. CONCLUSIONS: In summary, these results implied that TPO and AOX1 could serve as novel biomarkers for the diagnosis and prognosis of pediatric PTC.
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BACKGROUND: Lymphatic malformations (LMs) are benign congenital malformations that stem from the abnormal development of the lymphatic vessels during early embryogenesis. Somatic PIK3CA gene mutations are conventional cause leading to LMs. Both macrocystic and microcystic LMs arise due to lymphatic endothelial cell-autonomous defects, depending on the time in development at which PIK3CA gene mutation occurs. Recent study finds a PIK3CA mutation in 79% of LMs. However, discovering new genetic events in this disease is crucial to identify the molecular mechanism of the pathogenesis and further develop new targeted therapies. RESULTS: Here, we initially performed whole-exome sequencing in six children with LMs to find a new causal gene. Somatic mutations in PIK3CA (c.1633G > A [p. E545K] and PIK3CD (c.1997T > C [p.L666P]) were discovered in two different individuals. In vitro functional studies were conducted to demonstrate the pathogenicity of the novel mutation c.1997T > C in PIK3CD. We found that L666P promoted the cell proliferation and migration of human umbilical vein endothelial cells (HUVECs) and induced hyperactivation of the mTOR pathway. These findings indicate that the PIK3CD mutation affects downstream signalling in endothelial cells, which may impair normal lymphangiogenesis. CONCLUSIONS: This study reveals a novel candidate gene associated with the development of LMs, which is consistent with previous researches. These findings in our study may offer a novel gene target for developing therapies, which acts in tight interaction with the previously known PIK3CA.
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Fosfatidilinositol 3-Quinasa Clase I , Anomalías Linfáticas , Vasos Linfáticos , Niño , Fosfatidilinositol 3-Quinasa Clase I/genética , Células Endoteliales , Humanos , Anomalías Linfáticas/genética , Mutación/genética , Transducción de SeñalRESUMEN
OBJECTIVES: The objectives of this study were to explore the clinical heterogeneity of differentiated thyroid cancer (DTC) between prepubertal children and adolescents and guide clinical treatment. METHODS: A retrospective study included patients with DTC aged ≤19 years in Beijing Children's Hospital from June 2014 to June 2019. All patients were enrolled and divided into 2 subgroups based on the threshold age of 10 years, namely the childhood group (CG) (≤10 years old); and the adolescent group (AG) (between 10 and 19 years old). The χ2 test and Fisher's exact test were used to estimate the effect of risk factors in the 2 age groups. Multivariate binary logistic regression models were conducted to assess the recurrent risk factors. RESULTS: Seventy cases of DTC were included with an average age of 9.94 ± 2.88 years, including 35 in CG and 35 in AG. The most common clinical manifestation was a painless mass in the neck, accounting for 77.1% (54/70) of patients. Compared with the AG, the CG was more likely to have lymph node metastasis (p = 0.022) and distant metastasis (p = 0.041). The CG was more likely to have extrathyroidal extension (p = 0.012) and had a significantly higher recurrence rate than the AG (p = 0.040). Age was an independent variable predictive of recurrence (p = 0.0347). CONCLUSION: Regional invasiveness, cervical lymph node metastasis, and distant metastasis of DTC were more likely to occur in children ≤10 years old. Meanwhile, children ≤10 years old with DTC were more likely to have recurrence than adolescent's postsurgical treatment. Thus, children younger than 10 years of age with DTC should be treated more aggressively.
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OBJECTIVE/HYPOTHESIS: To evaluate the efficacy of initial sirolimus therapy in the treatment of intractable head and neck lymphatic malformations (LMs) in children. STUDY DESIGN: Prospective open-label study. METHODS: In this study, Twenty-seven children diagnosed with LMs were given oral sirolimus as primary treatment over a minimum 6-month trial. The major parameter to evaluate therapeutic outcome was percentage of lesion volume change compared with baseline. Average serum sirolimus concentrations, and adverse side effects, were monitored throughout the study period. RESULTS: Fifteen girls and twelve boys, average age 27 months (16 days-171 months), constitute the study group. Treatment was deemed effective for twenty-three participants, judged as fair in seven, good in nine, and excellent in seven. Two patients had minimal improvement, and two had increased volume to some degree. Effectiveness differed among LMs subtypes with responsiveness of macrocystic LMs exceeding that of microcystic LMs (P < .05). Adverse drug reactions totaled 27 events in ten patients, the majority being mild with upper respiratory infections being most common. CONCLUSIONS: Sirolimus as initial therapy is effective in decreasing lesion volume in intractable LMs in head and neck region, especially in macrocystic subtypes. Although most cases cannot be completely cured, side effects are few and tolerable. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1902-1908, 2021.
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Cabeza/anomalías , Anomalías Linfáticas/tratamiento farmacológico , Cuello/anomalías , Sirolimus/administración & dosificación , Administración Oral , Adolescente , Niño , Preescolar , Monitoreo de Drogas , Femenino , Humanos , Lactante , Recién Nacido , Anomalías Linfáticas/patología , Masculino , Estudios Prospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: Retropharyngeal lymphatic malformations (LMs) are uncommon congenital anomalies. Open surgery, sclerotherapy and various pharmaceutical agents have been described to manage these lesions. However, when such lesions involve the retropharyngeal space with evidence of airway compromise, none of the above aforementioned modalities has shown superior result without repeated imaging and procedures. We describe the use of radiofrequency ablation (Coblation) in an endoscopic-assisted, transoral approach as a one-off treatment for pediatric retropharyngeal lymphatic malformations. METHODS: Six patients with retropharyngeal lymphatic malformations, presented with airway compromise, were treated with endoscopic-assisted, transoral submucosal coblation in our unit between April 2015 to August 2018. We retrospectively reviewed their medical records, imaging and endoscopic findings. RESULTS: Five out of our six patients had LMs involving only the retropharyngeal space, who received the coblation as their sole treatment. One patient with extensive LMs involving multiple neck compartments received coblation to the retropharyngeal part as an adjunct procedure. The average operation time was 62.8 min, and the average intraoperative blood loss was 3.8 ml. All patients were safely extubated immediately after the surgery. None required admission to pediatric intensive care unit (PICU), nor prolonged dysphagia reported. They were observed for 2-5 days post operatively. The average follow up was 28 months (1-5 years) and none required re-operation to date. CONCLUSIONS: We presented our approach and outcome of 6 children with endoscopic-assisted, transoral submucosal coblation to retropharyngeal lymphatic malformation. We believe it is a safe and effective primary treatment to these patients, and a possible adjunct to complex vascular malformation involving multiple sites.
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Ablación por Catéter/métodos , Anomalías Linfáticas/cirugía , Enfermedades Faríngeas/cirugía , Preescolar , Endoscopía , Femenino , Humanos , Anomalías Linfáticas/diagnóstico por imagen , Masculino , Enfermedades Faríngeas/diagnóstico por imagen , Faringe/cirugía , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Malformaciones Vasculares/cirugíaRESUMEN
Papillary thyroid carcinoma (PTC) is the most common type of thyroid carcinoma, and its incidence has been on the increase in recent years. However, the molecular mechanism of PTC is unclear and misdiagnosis remains a major issue. Therefore, the present study aimed to investigate this mechanism, and to identify key prognostic biomarkers. Integrated analysis was used to explore differentially expressed genes (DEGs) between PTC and healthy thyroid tissue. To investigate the functions and pathways associated with DEGs, Gene Ontology, pathway and protein-protein interaction (PPI) network analyses were performed. The predictive accuracy of DEGs was evaluated using the receiver operating characteristic (ROC) curve. Based on the four microarray datasets obtained from the Gene Expression Omnibus database, namely GSE33630, GSE27155, GSE3467 and GSE3678, a total of 153 DEGs were identified, including 66 upregulated and 87 downregulated DEGs in PTC compared with controls. These DEGs were significantly enriched in cancer-related pathways and the phosphoinositide 3-kinase-AKT signaling pathway. PPI network analysis screened out key genes, including acetyl-CoA carboxylase beta, cyclin D1, BCL2, and serpin peptidase inhibitor clade A member 1, which may serve important roles in PTC pathogenesis. ROC analysis revealed that these DEGs had excellent predictive performance, thus verifying their potential for clinical diagnosis. Taken together, the findings of the present study suggest that these genes and related pathways are involved in key events of PTC progression and facilitate the identification of prognostic biomarkers.
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The aims of the present study were to reveal the prevalence of the TERT C228T mutation in pediatric papillary thyroid carcinoma (PPTC) and to further investigate the role of the TERT C228T mutation in PPTC. We also tested another TERT mutation, TERT C250T, although this was not detected in PPTC patients. In this study, 48 patients with PPTC (41 with classic PPTC) were enrolled. DNA was extracted from PPTC tissues and TERT C228T mutation analysis was performed. Chi-squared analysis, Fisher's exact test, and a t-test were applied to test the significance of differences. The TERT C228T mutation presented in 13 (27.1%) of the 48 PPTC patients and 10 (24.4%) of the 41 classical PPTC patients. There were significant differences between PPTC patients with the TERT C228T mutation and those without in terms of modified radical neck dissection, multifocality, capsular invasion, extrathyroidal invasion, and American Joint Committee on Cancer (AJCC) tumor stage (P<0.05). In classical PPTC, there were additional significant differences in other clinic-pathological features, such as AJCC nodal stage (P=0.009) and American Thyroid Association (ATA) PPTC stage (P=0.021) between patients with and without the TERT C228T mutation. These findings indicate that the TERT C228T mutation is significantly correlated with certain aggressive clinic-pathological features of PPTC.
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Telomerasa/genética , Cáncer Papilar Tiroideo/genética , Neoplasias de la Tiroides/genética , Adolescente , Niño , Preescolar , Análisis Mutacional de ADN , Femenino , Estudios de Asociación Genética , Humanos , Masculino , Mutación , Invasividad Neoplásica , Estadificación de Neoplasias , Regiones Promotoras Genéticas , Serogrupo , Telomerasa/metabolismo , Cáncer Papilar Tiroideo/clasificación , Neoplasias de la Tiroides/clasificaciónRESUMEN
Background: Lymphatic malformations (LMs) are caused due to abnormal lymphatic development, and mainly occur in neonates or young children. At present, the role of surgery in the treatment of head and neck LMs is still controversial, focusing mainly on surgical efficacy and indications. Objective: This study aimed to explore the effect and influential factors of surgical treatment in children with head and neck LMs, hoping to provide a basis for rational selection of surgical indications. Methods: This retrospective study enrolled 128 children with head and neck LMs and underwent surgical treatment in Beijing Children's Hospital from May 2007 to June 2016. They were classified into three morphological groups: macrocystic, microcystic, and mixed. Based on de Serres staging, they were divided into five groups: stage I to V. The local lesion control rate, complication rate, and recurrence rate were summarized and analyzed. Results: The rate of completely controlled and almost completely controlled in cases with head and neck LMs was 71.1%. The postoperative complication rate was 13.3%, and the postoperative recurrence rate was 11.9%. Statistically significant difference was found for local lesion control and postoperative recurrence rates between different morphological and clinical staging groups. Furthermore, the complication rate showed a significant difference between different morphological groups, but not between clinical staging groups. Conclusions: Surgical resection in children with macrocystic, low-stage, or neck-limited LMs demonstrated better therapeutic effect, with fewer complications. However, the effect remained poor and had more complications for microcystic, diffused and high-stage patients. High stage and incomplete resection are considered as the main factors for postoperative recurrence. Current staging system for LMs has important predictive value in the prognosis of head and neck LMs. For LMs in posterior pharyngeal space, plasma ablation has certain advantages.
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Quistes/cirugía , Anomalías Linfáticas/cirugía , Niño , Preescolar , Femenino , Estudios de Seguimiento , Cabeza/cirugía , Humanos , Recién Nacido , Anomalías Linfáticas/diagnóstico por imagen , Masculino , Cuello/cirugía , Complicaciones Posoperatorias , Estudios Retrospectivos , UltrasonografíaRESUMEN
BACKGROUND: Wide dynamic range compression (WDRC) has been widely used in hearing aid technology. However, several reports indicate that WDRC may improve audibility at the expense of speech intelligibility. As such, a modified amplification compression scheme, named adaptive compression, was developed. However, the effect of compression strategies on speech perception in pediatric hearing aid users has not been clearly reported. PURPOSE: The purpose of the present study was to investigate the effect of adaptive compression and fast-acting WDRC processing strategies on sentence recognition in noise with Mandarin, pediatric hearing aid users. RESEARCH DESIGN: This study was set up using a double-blind, within-subject, repeated-measures design. STUDY SAMPLE: Twenty-six children who spoke Mandarin Chinese as their primary language and had bilateral sensorineural hearing loss participated in the study. DATA COLLECTION AND ANALYSIS: Sentence recognition in noise was evaluated in behind-the-ear technology with both adaptive compression processing and fast-acting WDRC processing and was selected randomly for each child. Percent correct sentence recognition in noise with fast-acting WDRC and adaptive compression was collected from each participant. Correlation analysis was performed to examine the effect of gender, age at assessment, and hearing threshold of the better ear on signal-to-noise ratio, and a paired-samples t test was employed to compare the performance of the adaptive compression strategy and fast-acting WDRC processing. RESULTS: The mean percentage correct of sentence recognition in noise with behind-the-ear technology with fast-acting WDRC and adaptive compression processing were 62.24% and 68.71%, respectively. The paired-samples t test showed that the performance of the adaptive compression strategy was significantly better than the fast-acting WDRC processing (t = 3.190, p = 0.004). CONCLUSIONS: Compared with the fast-acting WDRC, adaptive compression provided better sentence recognition in noise for Mandarin pediatric hearing aid users.
Asunto(s)
Audífonos , Pérdida Auditiva Bilateral/fisiopatología , Pérdida Auditiva Bilateral/rehabilitación , Pérdida Auditiva Sensorineural/fisiopatología , Pérdida Auditiva Sensorineural/rehabilitación , Lenguaje , Ruido , Percepción del Habla , Adolescente , Niño , Preescolar , Método Doble Ciego , Femenino , Humanos , MasculinoRESUMEN
OBJECTIVE: To evaluate the efficacy of CO2 laser cauterization with suspension microlaryngoscopy as a definitive surgical treatment for pediatric Congenital Pyriform Sinus Fistula (CPSF). MATERIAL AND METHODS: This is a cohort retrospective study. Thyroid function and cervical ultrasonography examinations were performed before operation. Enhanced magnetic resonance imaging (MRI) was performed on patients with a repeated infection (≥2 times) and/or if they had a prior open surgery. Patients were divided into two groups: the <8-year-old group and the ≥8-year-old group. The differences in the number of cauterization procedures between the two age groups and between the initial treatment and the retreatment groups were analyzed. RESULTS: CO2 laser cauterizations with suspension microlaryngoscopy were performed for 104 CPSF patients. No complications occurred. Three patients had a recurrence in the follow-up. The number of surgical cauterization operations was fewer than 3 in 85.1% of the patients. There was no significant difference in the number of cauterizations among the different age groups or between the initial treatment and retreatment groups (P>0.05). CONCLUSION: CO2 laser cauterization with suspension microlaryngoscopy is a safe, effective, and minimally invasive approach to CPSF with optimal patient outcomes. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.