Pediatric Investigation最新文献

Importance: The diversity of pediatric genitourinary malignancies requires a timely resource detailing tumor characteristics and survival.

Objective: To determine the incidence, demographics, and outcomes of all pediatric genitourinary tumors within the United States.

Methods: A population-based search for patients diagnosed with genitourinary cancers under age 15 was performed using the National Cancer Institute's Surveillance, Epidemiology, and End Results 18 registry. Information on primary tumor location, histologic type, patient age, sex, year of diagnosis, race, treatment, cause of death, and survival months was extracted. Descriptive epidemiological and survival statistics were calculated for all variables.

Results: A total of 4576 cases from 1973 through 2015 were identified. The most common primary tumor sites were the kidney (80.3%), testis (12.3%), bladder (2.8%), and vagina (1.5%). Nephroblastoma (87.9%) and sarcoma (3.4%) were the most common renal malignancies. Rhabdomyosarcoma was common in the vagina, bladder, and testis at rates of 66.2%, 61.2%, and 24.6%, respectively. Germ cell tumors (71.0%) were the most common primary tumor of the testis. Ten-year overall survival (OS) for renal nephroblastoma and sarcoma was 88% and 82%, respectively. Ten-year OS for RMS of the testis was 91%, the bladder was 79%, the vagina was 79%, and the prostate was 56%. Germ cell tumor 10-year OS were 96% in the testis and 100% in the vagina.

Interpretation: A better understanding of the overall distribution and outcomes associated with pediatric genitourinary cancers allows physicians to best understand the patient's disease in the context of current frequency in a genitourinary setting and reported outcomes.

Importance: Cadherin-11 (CDH11), a cell-to-cell adhesion molecule, is implicated in the fibrotic process of several organs. Biliary atresia (BA) is a common cholestatic liver disease featuring cholestasis and progressive liver fibrosis in children. Cholestatic liver fibrosis may progress to liver cirrhosis and lacks effective therapeutic strategies. Currently, the role of CDH11 in cholestatic liver fibrosis remains unclear.

Objective: This study aimed to explore the functions of CDH11 in cholestatic liver fibrosis.

Methods: The expression of CDH11 in BA livers was evaluated by database analysis and immunostaining. Seven BA liver samples were used for immunostaining. The wild type (Wt) and CDH11 knockout (CDH11^-/- ) mice were subjected to bile duct ligation (BDL) to induce cholestatic liver fibrosis. The serum biochemical analysis, liver histology, and western blotting were used to assess the extent of liver injury and fibrosis as well as activation of transforming growth factor-β (TGF-β)/Smad pathway. The effect of CDH11 on the activation of hepatic stellate cell line LX-2 cells was investigated.

Results: Analysis of public RNA-seq datasets showed that CDH11 expression levels were significantly increased in livers of BA, and CDH11 was correlated with liver fibrosis in BA. BDL-induced liver injury and liver fibrosis were attenuated in CDH11^-/- mice compared to Wt mice. The protein expression levels of phosphorylated Smad2/3 were decreased in livers of CDH11^-/- BDL mice compared to Wt BDL mice. CDH11 knockdown inhibited the activation of LX-2 cells.

Interpretation: CDH11 plays an important role in cholestatic liver fibrosis and may represent a potential therapeutic target for cholestatic liver disease, such as BA.

The clinical outcomes of adolescents with avoidant/restrictive food intake disorder (ARFID) remain unclear. Furthermore, no report has compared the characteristics of ARFID and restricting-type anorexia nervosa (R-AN) in elementary-school students on total parenteral nutrition (TPN). This study retrospectively reviewed inpatients diagnosed with ARFID or R-AN between 2005 and 2019. Patients with ARFID (two boys and seven girls) and R-AN (13 girls) were hospitalized because of rapid physical deterioration, and nutrition therapy was continued without withdrawal. The ARFID group exhibited significantly lower body weights at admission than the R-AN group and gained an average of 6.5 kg during hospitalization; furthermore, the monthly weight gain during hospitalization was significantly higher, and no relapse was observed. Early physical improvement in ARFID resulted in good recovery. In conclusion, TPN can be easily introduced to patients with ARFID, in whom aversive eating is a concern, and is a suitable treatment for ARFID.

Importance: Children with 5-alpha-reductase deficiency (5α-RD) and hypospadias present with micropenis, which makes it difficult to obtain sufficient tissue for urethral reconstruction.

Objective: We investigated the therapeutic effects of oral testosterone undecanoate and established a standard androgen treatment protocol for patients with 5α-RD with micropenis.

Methods: Patients with 5α-RD were treated with oral testosterone undecanoate for 3 months as a course. All patients were treated with no more than 3 courses. If the penile length (PL) reached 2.5 cm (the minimum criterion for surgery) or greater than or equal to -2.5 standard deviations (SDs) (lower limit of normal), testosterone undecanoate was considered to be effective.

Results: The median age of 90 patients with 5α-RD was 1.7 years (0.9, 3.1 years). The baseline PL was 1.9 ± 0.6 cm before treatment. At the end of the first course, the PL of 63 patients (70%) reached 2.5 cm, and 49 patients (54%) reached greater than or equal to -2.5 SDs. After two treatment courses, the PL of 81 patients (90%) reached 2.5 cm, and 90 patients (100%) reached greater than or equal to -2.5 SDs. After three courses, the PL of all patients reached 2.5 cm, and all patients reached a PL greater than or equal to -2.5 SDs. No abnormal increase was observed in height-SD score, weight-SD score, or ratio of bone age to chronological age during the 1-3-year follow-up.

Interpretation: After 3-9 months of treatment, PL increased to the target length. No severe adverse reactions were observed during follow-up. Testosterone undecanoate was safe and effective in children with 5α-RD with micropenis.

Importance: Several studies conducted worldwide (mostly in Western countries) highlighted the negative effects of problematic internet use, particularly among adolescents aged 12 to 17, including depression, impulsivity, aggression, and social fear and avoidance. In Lebanon, literature on the prevalence and impact of problematic internet use among adolescents is limited.

Objective: This study aim was to study the association between problematic internet use and depression, impulsivity, anger, aggression and social phobia among Lebanese adolescents.

Methods: A cross-sectional study was performed on 1103 young adolescents (14-17 years), recruited from October 2017 till April 2018. The Internet Addiction Test (IAT) was used to evaluate the level of problematic internet use. Data were analyzed using the MANCOVA analysis. The main independent variable of interest was the IAT, while the dependent variables included the psychological scales.

Results: The multivariate analysis taking the psychological scales as the dependent variables and the problematic internet use (IAT score) as an independent variable, showed that problematic internet use was associated with higher depression, impulsivity, aggression, anger, hostility and social anxiety.

Interpretation: Problematic internet use has become an important health issue that should not be overlooked, particularly because of the increased use of the internet by adolescents. Educational programs on early exposure to the internet should be developed.

Introduction: Primary central nervous system lymphoma (PCNSL) is extremely rare in pediatric population. We reported a case of PCNSL in a 3-year-old girl and reviewed the literature in the past three decades.

Case presentation: A 3-year-old girl presented with gait disturbance. A contrast-enhanced magnetic resonance image of the brain showed a solitary bulky mass in the left cerebellar hemisphere, hydrocephalus and cerebellar tonsillar hernia. Surgical resection was performed and the patient was diagnosed with primary central nervous system lymphoblastic B cell lymphoma. Then the patient received regular chemotherapy, including 6 cycles of chemotherapy containing high-dose methotrexate (HD-MTX). The patient remains alive 15 months after the diagnosis with no evidence of active disease, but suffered twice chronic subdural hematoma, which was treated by burr hole drainage.

Conclusion: Lymphoblastic B cell lymphoma is a rare histologic subtype of pediatric PCNSL. Chemotherapy containing HD-MTX remains the most effective treatment. The patient should avoid head impact after surgical resection of the tumor to prevent chronic subdural hematoma.