Frontiers in Pediatrics最新文献

Background: Laparoscopic pyeloplasty is a minimally invasive approach for the therapy of infant ureteropelvic junction obstruction (UPJO), reliant on CO₂ pneumoperitoneum insufflation. While the impact of CO₂ insufflation on adult and older pediatric populations has been studied, its effects on infants remain less explored.

Methods: This prospective randomized controlled trial included infants with UPJO undergoing laparoscopic pyeloplasty. Patients were allocated to low pneumoperitoneum pressure (LPP, 5 mmHg) or high pneumoperitoneum pressure (HPP, 8 mmHg) groups. Surgical parameters, postoperative complications, acid-base balance, stress markers, inflammatory cytokines, and oxidative stress markers were evaluated and compared.

Results: A total of 116 infants were analyzed. Preoperative characteristics were comparable between LPP and HPP groups. No significant differences in blood loss, operation time, or hospitalization time were observed. Postoperative complications were similar between groups. Acid-base balance analysis revealed a decrease in pH after pneumoperitoneum in both groups, with greater reductions in actual base excess and standard base excess in the HPP group. Stress markers, cytokines, and oxidative stress markers increased postoperatively in both groups, with higher levels in the HPP group.

Conclusion: HPP leads to more pronounced physiological responses, including acid-base alterations, stress reactions, and inflammatory cytokine elevations.

Urticarial vasculitis (UV) is a type III hypersensitivity reaction, characterized by immune complex deposition in small vessels leading to complement activation. Hypocomplementemic urticarial vasculitis syndrome (HUVS) represents the most severe form of UV, manifesting as chronic and recurrent urticarial skin lesions with leukocytoclastic vasculitis on histology, hypocomplementemia, anti-C1q antibodies, and systemic organ involvement. This case study focuses on an adolescent who initially presented with invasive pneumococcal infection and was later diagnosed with two rare disorders: HUVS and coexisting complement factor 1 (CF1) deficiency by genotyping. The role of CF1 deficiency in the development of HUVS in this patient is uncertain but has not previously been described.

Aim: To compare volume-driven and cue-based feeding of low birth weight preterm infants, regarding short-term outcomes, including transition to oral feeds, weight gain, and length of stay.

Methods: This was a retrospective cohort study. Feeding and weight gain outcomes were compared between infants fed by volume-driven and cue-based feeds. The groups were subdivided by birth weight categories.

Results: The study group included 240 low birth weight preterm infants born before 34 weeks of gestation, 120 infants fed by volume-driven feeding were compared to 120 infants fed by cue-based feeding. The groups were sub-analyzed by birth weight categories: <1,500 g and 1,500-2,500 g. Study groups were comparable regarding baseline characteristics and neonatal morbidities. Infants fed by cue-based feeding were more likely to achieve full oral feeding faster and at an earlier gestational age. Infants with a birth weight <1,500 g were less likely to experience adverse respiratory episodes during cue-based feeding. Although the rate of weight gain was reduced in cue-based feeding in the heavier infant group, discharge weight, breastfeeding rates, and length of stay were comparable between the groups.

Conclusions: Cue-based feeding results in faster transition to full oral feeding in very low birth weight preterm infants and at an earlier gestational age.

Plasminogen deficiency type 1 (PLGD-1, hypoplasminogenemia) is an ultra-rare, lifelong disease associated with development of fibrinous lesions in multiple organ systems. Depending on lesion location, clinical manifestations of PLGD-1 can result in acute and/or chronic respiratory airway disease which can compromise respiratory function leading to life-threatening events. Early recognition and effective treatment of airway obstruction caused by fibrinous lesions are critical to prevent morbidity due to respiratory compromise. However, physicians may not be familiar with the clinical presentation and management of PLGD-1, causing delays in diagnosis and treatment and potentially contributing to morbidity. Presented here is a case series of one adult and three pediatric patients with severe respiratory complications of PLGD-1 successfully managed by infusions of plasminogen, human-tvmh replacement therapy. Patients' respiratory symptoms were resolved or greatly improved, and treatment was generally well tolerated. In all patients, baseline plasminogen activity was substantially increased with plasminogen replacement therapy administered initially every one to two days followed by extended interval dosing as symptoms were controlled or resolved. All four described cases support the clinical benefit of replacement therapy with plasminogen, human-tvmh in the resolution of life-threatening respiratory complications associated with PLGD-1. Clinical manifestations in addition to respiratory lesions were also improved or resolved with continued treatment.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a monogenic disorder characterized by multi-systemic autoimmunity secondary to loss-of-function mutations in the gene coding the forkhead box P3 (FOXP3) transcription factor which is important for the development, maturation, and maintenance of CD4 + regulatory T (T-reg) cells. Fewer than 300 affected individuals have been identified worldwide. The occurrence of IPEX is below 1:1,000,000. Herein we present a case of a 15-day-old male who was admitted to NICU 15 days after delivery due to respiratory distress. He was found to have metabolic acidosis due to DKA. During his stay in the NICU, he experienced seizures and was intubated for a month. He was diagnosed with neonatal diabetes. He also experienced recurrent respiratory infections and multiple episodes of diarrhea rash, and meningitis. At the age of 7 months, genetic testing confirmed IPEX with FOXP3 mutation, specifically the p.(Pro75Leu) variant of the FOXP3 gene. Subsequently, multiple family members were diagnosed. The unique variability observed in organ involvement and presentation timing among individuals within the same family, despite carrying an identical mutation, is a distinctive aspect, particularly considering the monoallelic expression of the FOXP3 gene in males. This phenomenon strongly suggests the presence of modifying genes that play a significant role in the pathogenesis of IPEX syndrome. The case presentation underscores the importance of clinical suspicion of IPEX in cases of neonatal DM. It also highlights the challenges associated with managing rare genetic disorders in pediatric patients. It also emphasizes that the IPEX genotype has a wide phenotype. This case is considered the first documented case of IPEX in Palestine.

Purpose: Analyze the clinical manifestations, laboratory tests, and imaging data of testicular torsion to provide clinical insights for timely and accurate diagnosis and treatment of testicular torsion.

Methods: A retrospective analysis was conducted on the clinical data of 67 pediatric patients suspected of testicular torsion, admitted and subjected to surgical exploration from June 2018 to June 2023. Based on whether the torsed testicle was excised during surgery, the patients were divided into orchiectomy group (40 cases) and orchidopexy group (27 cases). Combining clinical symptoms, signs, ultrasound examinations, and laboratory tests, the study aimed to summarize the influencing factors on the onset, diagnosis, and treatment of testicular torsion.

Results: The clinical manifestations of all 67 pediatric patients were generally typical. Color Doppler Flow Imaging (CDFI) and surgical exploration were performed for all cases, and the results were consistent. Testicular color doppler ultrasound suggested reduced or absent blood flow, leading to surgical treatment in all cases. All patients had unilateral testicular torsion, with 46 cases (68.66%) on the left side and 21 cases (31.34%) on the right side. Intrafunicular torsion occurred in 60 cases (89.55%), while extrafunicular torsion occurred in 7 cases (10.45%). The onset distribution was as follows: 20 cases in spring, 16 cases in summer, 16 cases in autumn, and 15 cases in winter. Univariate analysis indicated significant statistical differences in age, degree of testicular torsion, duration of symptoms, NEUT, NLR, and occurrence of tunica fluid between the two groups of patients. Multivariate logistic regression analysis showed that the duration of symptoms and the occurrence of hydrocele were independent risk factors for determining testicular viability.

Conclusion: Testicular torsion is more common in children and adolescents, with clinical manifestations including scrotal pain, scrotal redness and swelling, abdominal pain, nausea, and vomiting. In the early stages of testicular torsion, inflammatory markers in the blood increase, and preoperative ultrasound indicates hydrocele. This suggests that the testicle is in an early twisted state, with good viability and potential for preservation.

Background and objective: Physical activity (PA) is paramount for childhood development and growth. However, children diagnosed with Cerebral Palsy (CP) were often considered sedentary, and their physical inactivity was associated with adverse health conditions and complications. Therefore, this study aimed to objectively describe and compare the PA levels and SB levels of children with and without CP of the same age group. It also studied the factors correlating with PA, SB, and step count per day in children with CP.

Subjects and methods: A cross-sectional study using a wrist-worn accelerometer was conducted. PA and SB were measured over seven consecutive days.

Results: Eighty-five children aged 6-12 years, consisting of 41 children with CP and 44 TD children, participated in this study with a mean age of 9.18 ± 1.95 and 8.45 ± 1.78 years, respectively. According to the gross functional measures, 53.6% of children with CP were classified as first classification. A significant amount of time was spent in SB and Light PA (LPA) by children with CP compared to TD children, and no significant differences were observed in moderate PA (MPA) or step count. Gender mainly affected MPA as girls spent more time in MPA than boys. The age, height, and weight of children with CP correlate significantly with SB. As children's age, height, and weight increase, SB increases. Additionally, children with higher weights have lower step counts per day.

Conclusion: This study showed that children with CP spend more time in LPA and SB than typically developed children. Therefore, concerted efforts are needed to encourage physical activity and reduce the sedentary lifestyle, to take into account the gender and anthropometric measures of children to enhance the quality of life among children with CP, and to consider gender and anthropometric measures of the children.

Background: bacterial meningitis (BM) is more common in infants than at any other time in life and remains a devastating disease with considerable risk of death and morbidity. This article aims to gather the currently available evidence to perform a systematic review of clinical factors that may predict or be associated with BM death and/or sequelae in infants < 90 days of age.

Methods: The Medline/PubMed, Cochrane Library and Embase databases were systematically searched for prognostic studies that described risk factors for mortality and sequelae in infants aged <90d with BM. The databases were searched from the beginning of the database to December 31st, 2022.The quality of cohort studies was assessed by the Newcastle-Ottawa Scale (NOS). The quality of cross-section studies was assessed by the Agency for Healthcare Research and Quality (AHRQ). A systematic review was undertaken to ascertain the prognostic factors proven to be noteworthy.

Results: Of the 1,431 studies retrieved, 20 were eligible for the final analysis including 11 cohort and 9 cross-sectional studies were identified. Four risk factors predicting poor outcome were mentioned mostly in those studies, including prematurity or low birth weight (LBW), seizures, coma, and elevated CSF protein. But only preterm, coma and elevated CSF protein were identified by multivariate analyses in more than one study.

Conclusions: This study demonstrates several potential predictive factors to the poor outcomes of BM in infant. But with large heterogeneity, these predictors should be evaluated by further well-designed prospective studies.

Systematic review registration: https://www.crd.york.ac.uk/, identifier CRD42017074949.

Objectives: Understanding the characteristics of the pubertal growth spurt in Korean children and adolescents can serve as crucial foundational data for researching puberty and growth-related disorders. This study aims to estimate the key parameters of pubertal growth, specifically the age and magnitude of the pubertal growth spurt, utilizing longitudinal data from a cohort of Korean children and adolescents.

Methods: This study used mixed longitudinal height data from a cohort of Korean elementary, middle, and high school students aged 7-18 years. The Superimposition by Translation and Rotation (SITAR) model, a shape-invariant growth curve model, was utilized to estimate a reference height velocity curve for the entire dataset and individual curves via random effects to evaluate pubertal growth parameters. Altogether, 3,339 height measurements (1,519 for boys and 1,820 for girls) from 270 individuals (123 boys and 147 girls) were analyzed.

Results: The average age of growth spurt onset in Korean boys was 10.17 ± 0.61 years (mean ± SE), with peak height velocity occurring at 12.46 ± 0.69 years of age (9.61 ± 1.26 cm/year). Korean girls, contrarily, experience their growth spurt at an earlier age (8.57 ± 0.68 years), with peak height velocity occurring at 10.99 ± 0.74 years of age (8.32 ± 1.09 cm/year). An earlier onset of puberty in both sexes is associated with a shorter growth spurt duration (0.63 years for boys and 0.58 years for girls) and a higher peak height velocity (1.82 cm/year for boys and 1.39 cm/year for girls). These associations were statistically significant for both sexes (all p < 0.0001).

Conclusion: This study is the first to use the height velocity curve from the SITAR model to examine the pubertal growth spurt of Korean children and adolescents. The estimated timing and magnitude of the pubertal growth spurt, and their relationships can be useful data for clinicians and researchers.

Introduction: Impaired future fertility potential secondary to gonadotoxic therapies for childhood cancer is a shattering aftermath faced by childhood cancer survivors. Fertility preservation (FP) has emerged as a key to mitigate this unwelcomed sequelae. FP services catering to the needs of children and adolescents (C&A) population in developing countries are limited. Malaysia recently launched its pioneering pediatrics FP services.

Aims of study: To evaluate healthcare providers' (HCPs) FP knowledge, practice behaviors, attitudes, perceptions, and barriers towards FP counseling/services (C/S) for the C&A cohort.

Methods: A questionnaire-based study was conducted utilizing a questionnaire consisting of 51 items which was adapted from G.Quinn et al. The questionnaire was distributed both online and physically amongst HCPs in a tertiary center. Ethical committee approval was granted by the Research Ethical Committee, Universiti Kebangsaan Malaysia.

Results: A total of 102 HCPs completed the questionnaires. The majority of respondents were Malays (74.5%), females (80.4%), gynecology/pediatrics specialty (76.5%), and had children (88.2%). Nearly 72% of HCPs demonstrated good knowledge of FP. Almost 73% of HCPs consulted reproductive specialists (RES) on potential fertility issues and over 80% of HCPs referred patients who enquired on fertility issues to RES. Only 17% of HCPs practiced FP discussion, 12% reported no available person to discuss FP, and 10% of HCPs were unaware of who to discuss FP with. Patients' inability to afford FP (30.4%) tops the list of barriers to FP C/S, followed by limited available information on FP for patients (17.6%) and patients too ill to delay treatment (12.7%). Most HCPs (88.2%) demonstrated unfavorable attitudes towards FP C/S.

Discussions: In general, the majority of our HCP respondents demonstrated good current FP knowledge and practice behaviors. Mitigating several controversial issues in FP would improve HCPs' attitude towards FP. Main barriers to the uptake of FP C/S for C&A were patient and resource barriers. Addressing these issues by funding aid for FP procedures, increasing FP knowledge dispersion, as well as developing age-appropriate FP-related educational materials would improve FP service provision for C&A in the future.

Conclusions: In conclusion, successful corrective action combined with strategic planning points to a promising future for Malaysia's FP services provision for C&A.