Children's Medical Research Articles

Normal Portal Vein Diameter (PVD) and Peak Systolic Velocity (PSV) in Healthy Iranian Children

Background: It is crucial to determine the normal Portal Vein Diameter (PVD) in different populations, and ages since changes in the size of the vein are used as an index in the diagnosis of some diseases. Therefore, it is important to determine its normal size. In this regard, the present study aimed to investigate the PVD and Peak Systolic Velocity (PSV) in healthy Iranian children. Methods: The present descriptive-analytical study examined 250 healthy Iranian children who visited the Imaging Center of the Children Medical Center of Excellence. The PVD and PSV were examined by a radiologist using a Doppler ultrasound. Children were classified intofive age groups: under one month, 1 month to 2 years, 2 to 6 years, 6 to 10 years, and 10 to 18 years, and the above-mentioned indices were measured and compared in them. Statistical analysis was conducted using SPSS 26 employing the Mann-Whitney, Kolmogorov-Smirnov, and Kruskal-Wallis tests. Results: In children under one month, one month to 2 years, 2-6 years, 6-10 years, and 10-18 years of age, the mean values of PVD were 4.08, 5.64, 6.14, 7.50, and 8.32 mm, and the mean PSV values were 19.26, 22.20, 21.68, 22.86, and 21.48, respectively. The mean PVD and PSV increased with increasing age; however, no statistically significant difference was found between the mean values of the indices in both boys and girls (P = 0.18). Conclusions: The PVD and PSV in healthy Iranian children were relatively similar to their non-Iranian counterparts, and the mean values increased with age. Additionally, no significant difference was found between the mean values of PVD and PSV in boys and girls based on gender.

Longitudinal study on catch-up growth in preterm infants with small for gestational age at corrected ages 0-24 months

To understand the growth and development status and differences between small for gestational age (SGA) and appropriate for gestational age (AGA) preterm infants during corrected ages 0-24 months, and to provide a basis for early health interventions for preterm infants. A retrospective study was conducted, selecting 824 preterm infants who received regular health care at the Guangzhou Women and Children's Medical Center from July 2019 to July 2022, including 144 SGA and 680 AGA infants. The growth data of SGA and AGA groups at birth and corrected ages 0-24 months were analyzed and compared. The SGA group had significantly lower weight and length than the AGA group at corrected ages 0-18 months (P<0.05), while there were no significant differences between the two groups at corrected age 24 months (P>0.05). At corrected age 24 months, 85% (34/40) of SGA and 79% (74/94) of AGA preterm infants achieved catch-up growth. Stratified analysis by gestational age showed that there were significant differences in weight and length at corrected ages 0-9 months between the SGA subgroup with gestational age <34 weeks and the AGA subgroups with gestational age <34 weeks and 34 weeks (P<0.05). In addition, the weight and length of the SGA subgroup with gestational age 34 weeks showed significant differences compared to the AGA subgroups with gestational age <34 weeks and 34 weeks at corrected ages 0-18 months and corrected ages 0-12 months, respectively (P<0.05). Catch-up growth for SGA infants with gestational age <34 weeks and 34 weeks mainly occurred at corrected ages 0-12 months and corrected ages 0-18 months, respectively. SGA infants exhibit delayed early-life physical growth compared to AGA infants, but can achieve a higher proportion of catch-up growth by corrected age 24 months than AGA infants. Catch-up growth can be achieved earlier in SGA infants with a gestational age of <34 weeks compared to those with 34 weeks.

Long-Term outcomes of pediatric dacryocystorhinostomy in a single medical center- a retrospective study.

To examine the long-term success rate of pediatric endoscopic DCR surgery via telephone questionnaires, as determined by patients and their parents. This is a retrospective cohort study of all patients who underwent DCR surgery at the Schneider Children's Medical Center of Israel between 2010 and 2020. We performed long-term follow-ups to assess the quality of life, surgical complications, and satisfaction with surgical outcomes. Our study includes seventy-nine patients with a total of 108 eyes. The mean age at the time of DCR was 7.05 years (Std = 4, min = 0.3, max = 17.7) Mean follow-up time was 5.7 years (Std =2.5, min = 1.4, max = 11.1). Tubes were inserted for a mean of 129 days (Std = 101). Fifty-seven patients (72%) declared they had no complications after surgery, three patients (4%) reported pain after surgery, and 14 patients (17.7%) reported tube extrusion, which occurred 7-21 days after surgery. Forty-four patients (56%) reported no recurrence of symptoms, 29 (37%) complained of mild epiphora, and 18 (23%) reported some ocular discharge. Sixty-eight patients (86%) stated that they did not undergo additional surgery, while the remaining 11 (14%) reported undergoing a revision operation for symptom control. Satisfaction rate (1-7) mean score reported was 6.15 (Std = 1.6). Sixty-two (78%) reported improved quality of life, while 17 (22%) reported no improvement. Our questionnaire results have been compared with the TEARS scores with similar findings. Regardless of its etiology, endoscopic DCR surgery in the pediatric population is safe and efficient, with a high long-term patient satisfaction rate, as reported via a telephone questionnaire.

Ultrasonographic measurement of mediastinal shift angle for the prediction of postnatal survival in fetuses with isolated left congenital diaphragmatic hernia.

The main objective of the study was to determine if ultrasonographic measurement of mediastinal shift angle (MSA) can predict postnatal survival in fetuses with isolated left congenital diaphragmatic hernia (CDH). This relatively new technique may be used to enhance antenatal prediction of survival in fetuses with CDH. A retrospective cross-sectional study was conducted at the Philippine Children's Medical Center involving 16 cases with prenatally diagnosed isolated left CDH and 60 controls with normal fetuses. The cases with prenatally diagnosed isolated left CDH were allocated into two groups: Group A (survivors) and group B (non-survivors). For all fetuses (study and control groups), MSA was determined independently by two operators. The diagnostic capacity of MSA was assessed using the receiver operating characteristic curve. The mean MSA for the control group was 17.18°. Among CDH cases, the mean MSA was 33.04° and 37.57° for survivors and non-survivors, respectively. Results showed that MSA significantly predicted the probability of neonatal survival status (OR = 0.46, P = 0.021). The best cutoff score of MSA based on the receiver operating characteristic curve was 33.80° with 87.50% sensitivity and specificity. MSA has the potential to enhance antenatal prediction of survival in fetuses with CDH and may serve as a guide in prenatal counseling and evaluation of the need for in-utero treatment or postnatal procedures.

Prevalence and Resistance Profiles of Pediatric Enterococcal Isolates: A Five-Year Update from Children's Medical Center Hospital, Tehran.

In this study, attempts were made to evaluate the frequency of high-level gentamicin-resistant (HLGR) and vancomycin-resistant enterococci (VRE) and the prevalence and antibiotic resistance profile of enterococcal species isolated from pediatric patients referred to Children's Medical Center Hospital, Tehran, over five years. A total of 404 enterococcal isolates from different patients referred to the Children's Medical Center between March 2016 and March 2021 were included in this cross-sectional study. Antimicrobial susceptibility testing was performed using standard methods according to the guidelines of the Clinical Laboratories Standards Institute (CLSI). Approximately one-third of the enterococcal strains were isolated from urology and intensive care units. 17.3% of the isolates were obtained from outpatient sources. However, 82.7% of the isolates were sourced from inpatient settings. We found that the rates of resistance to ampicillin, penicillin, and vancomycin were twice as high in inpatients as in outpatients. Of the total isolates, 87.4% and 49.3% were identified as HLGR and VRE, respectively. In addition, we identified 2% of the VRE isolates that were not susceptible to linezolid. Nitrofurantoin showed excellent activity against enterococcal isolates in the urine, with a susceptibility rate of 92.5%. The present study reports the highest range of VRE isolated from pediatric patients in Iran. Despite the predominance of HLGR enterococci in our region, vancomycin remains effective against such strains. This study is among the few to demonstrate the incidence of linezolid-insensitive VRE in pediatric patients. Therefore, it is important to evaluate effective infection control measures to prevent linezolid and vancomycin resistance in enterococci.

Imaging features and clinical evaluation of pulmonary nodules in children.

With the widespread use of computed tomography (CT), the detection rate of pulmonary nodules in children has gradually increased. Due to the lack of epidemiological evidence and clinical guideline on pulmonary nodule treatment in children, we aimed to provide a reference for the clinical diagnosis and management of pediatirc pulmonary nodules. This retrospective study collected consecutive cases from April 2012 to July 2021 in the Shanghai Children's Medical Center. The sample included children with pulmonary nodules on chest CT scans and met the inclusion criteria. All patients were categorized into tumor and non-tumor groups by pre-CT clinical diagnosis. Nodule characteristics between groups were analyzed. To establish a clinical assessment model for the benign versus malignant pulmonary nodules, patients who have been followed-up for three months were detected and a decision tree model for nodule malignancy prediction was constructed and validated. The sample comprised 1341 patients with an average age of 7.2 ± 4.6 years. More than half of them (51.7%) were diagnosed with malignancies before CT scan. 48.3% were diagnosed with non-tumor diseases or healthy. Compared to non-tumor group, children with tumor were more likely to have multiple nodules in both lungs, with larger size and often be accompanied by osteolytic or mass lesions. Based on the decision tree model, patients' history of malignancies, nodules diameter size≥5mm, and specific nodule distribution (multiple in both lungs, multiple in the right lung or solitary in the upper or middle right lobe) were important potential predictors for malignity. In the validation set, sensitivity, specificity and AUC were 0.855, 0.833 and 0.828 (95%CI: 0.712-0.909), respectively. This study conducted a clinical assessment model to differentiate benignity and malignancy of pediatric pulmonary nodules. We suggested that a nodule's diameter, distribution and patient's history of malignancies are predictable factors in benign or malignant determination.

Regular prophylaxis with activated prothrombin complex concentrates in pediatric hemophilia.

Regular prophylaxis with activated prothrombin complex concentrates (aPCCs) is effective in adult patients with hemophilia with inhibitors; however, data in children are scarce. This was a single-center retrospective study at Saitama Children's Medical Center. Patients with severe and moderate hemophilia with inhibitors aged <15 years at the start of aPCCs prophylaxis were included. Medical records were retrospectively reviewed. We treated nine pediatric patients with hemophilia with inhibitors (median age, 1.9 years; age range, 1.3-12.9 years; inhibitor titers before treatment with aPCCs, 5.9-69 BU/mL) using prophylactic aPCCs (doses, 50-100 U/kg; 2-3 times/week). The median prophylactic period was 13 months (range: 5-31 months). The median annualized bleeding rate (ABR) during prophylactic treatment with aPCCs was 2 (range, 0-17). In four patients, ABR was reduced by 19%-100% with prophylactic aPCCs compared to on-demand aPCCs. An adverse effect of treatment was that a patient with hemophilia B developed nephrotic syndrome 34 months after starting regular prophylaxis with aPCCs. Regular prophylactic aPCCs reduced the ABR even in younger children with hemophilia A and B. Serious adverse events include nephrotic syndrome, which requires caution.

Success rate and predictors of failure of enema reduction of intussusception in children with a water-soluble contrast medium at a height of 120 cm or less.

This study investigates the reduction rate and failure predictors of hydrostatic enema reduction for intussusception. The procedure typically begins with a water-soluble contrast medium at 90 cm above the patient, subsequently elevated to 120 cm. Our focus is on the reduction rate during initial attempts when the contrast medium is positioned at or below 120 cm from the patient. Hydrostatic enema reductions for intussusception, performed between March 2010 and May 2022 at Tokyo Metropolitan Children's Medical Center, were investigated retrospectively. The initial attempts involved one or more trials. The clinical characteristics, treatment modalities, and outcomes were analyzed. Logistic regression was used to identify the predictors of failure when the reduction was performed with the water-soluble contrast medium at a height of 120 cm or less. Reduction was achieved successfully with the water-soluble contrast medium at a height at or below 120 cm in 77.5% of 351 patients. When reductions performed at heights greater than 120 cm were included, 333 (94.9%) were successful during the initial attempts and were unaccompanied by complications. Predictors of failure of reductions performed at or below 120 cm were age less than 12 months and the presence of trapped fluid. The present study found a successful reduction rate of 77.5% during the initial attempts, suggesting that a height greater than 120 cm may yield an even greater success rate. Children aged below 12 months and those with trapped fluid may have a greater failure risk during the initial attempts.

The Value of Long-term Video EEG Monitoring to Diagnose and Track Childhood Epilepsy.

Long-term video-EEG monitoring (LTM) is a new technique to assess and track fluctuations, classify seizures, identify epileptic syndromes, and determine the number of seizures and epilepsy-simulating disorders. The present study aims to evaluate the concordance of traditional EEG and LTM in assessing childhood epilepsy. This cross-sectional before-after study was performed on 120 children with epilepsy who were referred to the Epilepsy Monitoring Unit (EMU) at the Children's Medical Center between September 2021 and September 2022 and were monitored for at least eight hours in this unit. The source of the study information collection was the patients' recorded files. A neurologist reviewed the primary EEGs, and two experts blindly reviewed and interpreted the patients' LTMs under a clinical neurophysiologist's supervision. The diagnoses changed after employing LTM in most children with epilepsy. Based on the diagnostic agreement analysis between EEG and LTM, the coefficient value for LTM was calculated at -0.37 (p= 0.229), showing that LTM has significantly expanded patients' diagnoses and care plans. The use of LTM improves the diagnosis, classification, and monitoring of epilepsy in affected children and can be a reliable supplement to EEG in some instances.

Development, Reliability and Validity of the Medication Literacy Scale for Parents of Children with Epilepsy.

This study aimed to develop a medication literacy scale for parents of children with epilepsy (MLSPCE) and to test the reliability and validity of the scale. The pilot scale was formulated based on the concept of medication literacy, the knowledge-attitude-practice model, and relevant literature reviews. It was formed through two rounds of expert consultations using the Delphi method. A survey of 657 parents of children with epilepsy, who were admitted to the neurology department or examined in the neuro-electrophysiological outpatient department of Guangzhou Women and Children Medical Center, using the pilot scale was conducted from October 2021 to January 2022 to test the reliability and validity of the scale questionnaire. The content validity of the scale questionnaire was assessed by consulting 20 neurology nursing, neurology clinician, and nursing education experts. Numbers, percentages, t-test, correlation analysis, Cronbach's alpha reliability coefficient and factor analysis were used for data analysis. The MLSPCE included 34 items in four dimensions. Ten factors were drawn from the explorative factor analysis, with a cumulative variance contribution rate of 62.32%. The content validity index of each item on the 34-item scale was between 0.81 and 1.0, and the scale-content validity index/ average was 0.97. The correlation coefficient between each item and its dimension was between 0.399 and 0.760, the correlation coefficients between dimensions were between 0.150 and 0.382, and the correlation coefficients between each dimension and the total scale were between 0.390 and 0.845. Differences for all comparisons were statistically significant (P < 0.05). Cronbach's alpha coefficient for the total scale was 0.864, and the split-half reliability of the total scale was 0.923. All the statistical procedures performed in the validity and reliability stages of the study showed that MLSPCE is a valid and reliable tool for measuring medication literacy among Chinese parents of children with epilepsy.

Prevalence of Anatomical Anomalies in Pediatric Patients Hospitalized with UTIs in a Tertiary Care Center's Urology and Nephrology Ward

Background: Urinary tract infections (UTIs) are a significant pediatric health concern, often linked to anatomical anomalies of the urinary tract. Understanding the prevalence and characteristics of these anomalies in children with UTIs is crucial for effective diagnosis and management. Objective: This study aimed to investigate the prevalence and nature of anatomical anomalies in children diagnosed with UTIs, focusing on age and gender distribution, and to identify common anomalies associated with UTIs in pediatric patients. Methods: A retrospective cross-sectional study was conducted at the Children's Medical Center in Tehran, from March to September 2022. The study included pediatric patients admitted with UTIs, excluding those over 18 years of age. Convenience sampling was used to target at least 70 patients with definitive anatomical anomalies. Data collection involved reviewing medical records and laboratory results, focusing on variables such as age, sex, paraclinical tests, and types of anatomical anomalies. Statistical analysis was performed using SPSS software version 25, employing Fisher’s exact or χ2 tests for categorical variables, and the student’s t-test, ANOVA, and Chi-Square tests for continuous variables. Results: The average age of patients was 29.61 months (SD = 35.28), with a median of 12.50 months (IQR = 36). The average number of UTI episodes was 3.43 (SD = 2.31), ranging from 1 to 10 episodes. Bilateral hydronephrosis was observed in 15 patients (21.7%), while neurogenic bladder and Reflux Grade 4 or 5 were identified in 11 (15.9%) and 14 (20.3%) patients, respectively. VCUG was performed in 63 patients (91.3%). Unilateral hydronephrosis was the most common imaging finding, observed in 33 patients (47.8%). Conclusion: The study concludes that children around 29 months of age, particularly females, are more prone to have anatomical anomalies associated with UTIs. Bilateral hydronephrosis was a prevalent anomaly. However, limitations such as potential data unavailability or misinterpretation, errors in laboratory data, and patient comorbidities must be considered when interpreting these findings.

Brain magnetic resonance imaging as predictors in pediatric anti-N-methyl-D-aspartate receptor encephalitis

ObjectiveTo investigate the associations between brain magnetic resonance imaging (MRI) changes and clinical profiles in children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. MethodsClinical data and brain MRI results of children diagnosed with anti-NMDAR encephalitis in Guangzhou Women and Children's Medical Center from October 2014 to June 2022 were retrospectively studied. ResultsA total of 143 children (Male: female 54:89) were enrolled, with a mean onset age of 6.8 years (6.8 ± 3.1). 40.6 % (58/143) of patients had abnormal initial brain MRI. Lesions in temporal lobe (34.5 %, 20/58) and frontal lobe (25.9 %, 15/58) were relatively common. Children with abnormal initial brain MRI were prone to have fever (P = 0.023), dystonia (P = 0.037), positive MOG antibodies (P = 0.015), higher cerebrospinal fluid (CSF) white blood cell count (WBC) (P = 0.019) and to receive rituximab treatment (P = 0.037). There were no significant differences in modified Rankin Scale (mRS) scores before immunotherapy, after immunotherapy and at last follow-up between the normal initial brain MRI group and abnormal group. No initial brain MRI changes were found to be associated with relapses. Brain MRI was reviewed in 72 patients at last follow-up with a median follow-up time of 25.5 months and 48.6 % (35/72) of patients had abnormal brain MRI. The mRS score of the group with normal brain MRI at last follow-up was significantly lower than that of the abnormal group. ConclusionsAbout 40.0 % of children with anti-NMDAR encephalitis had abnormal initial brain MRI. Initial brain MRI was associated with certain clinical profiles, but not with relapse and prognosis. Around half of patients had abnormal brain MRI at last follow-up and were prone to have higher mRS score.

The relationship between demographic factors and syncopal symptom in pediatric vasovagal syncope

This research proposed to retrospectively analyze 20 years of clinical data and investigate the relationship between demographic factors and syncopal symptom in pediatric vasovagal syncope. A total of 2513 children, 1124 males and 1389 females, age range 3–18 years, who presented to Department of Pediatric Cardiovasology, Children's Medical Center, The Second Xiangya Hospital, Central South University with unexplained syncope or pre-syncope and were diagnosed with vasovagal syncope were retrospectively collected and divided into syncope group (n = 1262) and pre-syncope group (n = 1251). (1) Females had a 36% increased risk of syncope compared to males, a 27% increased risk of syncope for every 1-year increase in age, and a 2% decreased risk of syncope for every 1 cm increase in height. (2) A non-linear relationship between age, height, weight and syncope was observed. When age > 10.67 years, the risk of syncope increases by 45% for each 1-year increase in age; when height < 146 cm, the risk of syncope decreases by 4% for each 1 cm increase in height; when weight < 28.5 kg, the risk of syncope decreases by 10% for each 1 kg increase in weight. Demographic factors are strongly associated with syncopal symptom in pediatric vasovagal syncope and can help to predict the risk.

Long-term outcome of hematopoietic stem cell transplantation in two children with Mucopolysaccharidosis

To explore the long-term efficacy of allogeneic hematopoietic stem cell transplantation (alloHSCT) in patients with Mucopolysaccharidosis (MPS), which has rarely been reported in China. A 18-month-old boy and a 23-month-old girl undergoing alloHSCT for MPS VI and MPS IH Shanghai Children's Medical Center on March 30, 2006 and September 6, 2006 were selected as the study subjects. A busulfan-based myeloablative regimen was used as the conditioning regimen. Peripheral stem cells were respectively collected from a human leucocyte antigen (HLA) matched sibling carrier donor and a HLA 9/10 matched unrelated donor. Both patients were followed up for more than 15 years. The functions of internal organs before and after the transplantation were compared, and child 1 was also compared with his untreated brother and healthy brother. Both children have achieved full donor chimerism after the transplantation, and their enzymatic activities have remained stable. The enzymatic activity of the child 1 was slightly lower than normal but similar to that of his carrier donor, whilst that of the child 2 was normal. Both children have attended schools with good academic performance. Compared with his untreated brother, the respiratory function and hearing of child 1 have significantly improved. However, his orthopedic and cardiac disorders have still remained and required medical intervention. For child 2, her obstructive pulmonary disease was resolved and cognitive development was well preserved after the HSCT. Her heart disease has become stabilized and even improved with time, though her corneal clouding and skeletal malformation still required surgery. MPS patients can sustain long-term and stable enzymatic activities after successful alloHSCT. Compared with untreated patients, their health can be significantly improved, along with considerably prolonged survival, though the long-term efficacy of HSCT for different organs may vary to a certain extent.

Establishment of a children's Drugs and Therapeutics Committee to ensure evidence-based and cost-effective medical treatment for children.

The prevalence of undocumented medical treatments among children is a significant issue, as well as many EU countries lack access to newly developed children-friendly medicines. Consequently, there is a pressing need for supplementary resources that can facilitate informed decision-making regarding children's medication. We therefore aim to describe the process of establishing a children's Drug and Therapeutics Committee (cDTC), as well as the preparing and implementation of recommendations for children in the capital region of Denmark. Following the guidelines outlined by the World Health Organization, we established a cDTC, and recommendations for paediatric medication practice were constructed from assessments of medication use patterns among children in the capital region between 2019 and 2021. The recommendations were meticulously crafted based on evaluation of the current marketing authorization landscape and existing best available evidence. In 2019, the capital region established the first cDTC supported by expert councils and an editorial board. A total of 2429 purchase item numbers covering 1 222 846 defined daily doses and 592 088 purchased packages covering 10 200 000 defined daily doses were identified in the secondary and primary sectors, respectively. Three comprehensive lists covering recommendations for newborns and children were published between 2021 and 2020 totaling 331 recommended pharmaceutical products. The recommendations primarily intended for use in the secondary healthcare sector were implemented through the revision of 38 paediatric- and six neonatal product ranges throughout capital region. In conclusion, recommendation lists for children governed by a cDTC provide a rational auxiliary tool that can be immediately implemented in the clinic.

Autosomal dominant neurodevelopmental disorders associated with KIF1A gene variants in 6 pediatric patients.

To analyze the clinical and genetic characteristics of children with autosomal dominant neurodevelopmental disorders caused by kinesin family member 1A (KIF1A) gene variation. Clinical and genetic testing data of 6 children with KIF1A gene de novo heterozygous variation diagnosed in Shanghai Children's Medical Center Affiliated to Shanghai Jiao Tong University School of Medicine from the year 2018 to 2020 were retrospectively analyzed. Pathogenic variants were identified based on whole exome sequencing, and verified by Sanger sequencing. Moreover, the effect of variants on three-dimensional structure and stability of protein was analyzed by bioinformatics. Among 6 patients there were 4 males and 2 females, and the age of consultation varied from 7 months to 18 years. All cases had varying degrees of motor developmental delay since childhood, and 4 of them had gait abnormalities or fell easily. In addition, 2 children were accompanied by delayed mental development, epilepsy and abnormal eye development. Genetic tests showed that all 6 cases had heterozygous de novo variations of KIF1A gene, including 4 missense mutations c.296C>T (p.T99M), c.761G>A (p.R254Q), c.326G>T (p.G109V), c.745C>G (p.L249V) and one splicing mutation c.798+1G>A, among which the last three variants have not been previously reported. Bioinformatics analysis showed that G109V and L249V may impair their interaction with the neighboring amino acid residues, thereby impacting protein function and reducing protein stability, and were assessed as "likely pathogenic". Meanwhile, c.798+1G>A may damage an alpha helix in the motor domain of the KIF1A protein, and was assessed as "likely pathogenic". KIF1A-associated neurological diseases are clinically heterogeneous, with motor developmental delay and abnormal gait often being the most common clinical features. The clinical symptoms in T99M carriers are more severe, while those in R254Q carriers are relatively mild.

Outcomes after surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: a series of 104 cases

Objective: To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies. Methods: A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children's Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged (M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results: In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95%CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95%CI: 89.9% to 100%). Conclusions: Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

Psychological, socioeconomic burdens and coping mechanisms of caregivers of children with bronchoasthma in Kakamega County, Kenya

Background: Childhood bronchoasthma consistently pose diverse caregiving burdens yet, magnitude of these and coping mechanisms adopted by caregivers are infrequently assessed during routine care only focused on index patients. Methods: A partially mixed descriptive cross-sectional study was conducted for a minimum of 408 respondents. The results were presented in form of tables and text. Results: A total of 408 caregivers were surveyed, with a mean age of 33 years (SD=9.86). Majority (78.7%) were actual parents of whom 73.5% were married. Primary-level education was the highest attained by 64% of caregivers while those earning &lt;10,000 KES per month were 76% with about 3 out 4 being self-employed. Significant psychological concerns include sleep disturbance on the night previous to the interview, caregivers’ concern about the child's asthma medication and side effects. Work absenteeism; reduced total annual income; lost job; discontinued child schooling and delayed family investment/cut budgets were significant social/economic burdens. Most caregivers used escape-avoidance coping mechanisms with a significant majority being involved in substance abuse (72.3%); feeling helpless or downplayed the severity of symptoms because of myths and stigma. Young age (p=0.002); being widowed (p=0.040), and being of low level of education (p&lt;0.001) were associated with poor coping mechanisms. Conclusions: There was considerable prevalence of psychological burden and socio-economic status among caregivers with significant majority using adversarial coping mechanisms, particularly among socio-economically disadvantaged.

Parents' Perceptions Regarding Their Children's Medications and Expert-Assessed Drug-Related Problems in Pediatric Patients with Inborn Errors of Metabolism.

We aimed to explore parents' perceptions of their children's medication use for inborn errors of metabolism (IEM), including the importance of medication intake, potential complications, and concerns about adverse drug reactions (ADR). Additionally, we aimed to determine expert-assessed clinically relevant drug-related problems, particularly those attributable to IEM. We interviewed 108 parents of 119 pediatric patients with IEM using a questionnaire relating to their perceptions regarding their children's IEM medication. In affected siblings, a questionnaire was used for each child. We performed medication analyses to evaluate the patient's complete medication regimen for clinically relevant drug-related problems, including medication for conditions other than IEM. It was very important to the parents of 85% of the patients to use IEM medication exactly as prescribed. The parents of 41% of patients perceived complications in their children's use of IEM medication. The parents of 47% of patients reported fears concerning ADR because of IEM medication. Parents observed ADR in 27% of patients because of IEM medication. In 44% of patients, medication for conditions other than IEM was inadequate because of drug-related problems not associated with the IEM; a safe alternative existed in 21% of patients. In summary, almost half of the parents of patients with IEM reported complications with their child's IEM medication intake and fears of ADR. Medication analyses showed that drug-related problems occurred regardless of IEM, emphasizing the general need to prescribe and dispense adequate, child-appropriate medication to minimize clinically relevant drug-related problems in pediatric patients.

Vaccination Recommendations and Clinical Characteristics of Children after Allogeneic Hematopoietic Stem Cell Transplantation

To investigate the clinical characteristics, the risk factors related to antibody-negative conversion, and the effectiveness and safety of revaccination in children who underwent allogeneic hematopoietic stem cell transplantation (Allo-HSCT). The clinical characteristics of 516 children after Allo-HSCT who were eligible for revaccination in the Vaccination Evaluation Clinic of Shanghai Children's Medical Center from March 2017 to March 2023 were analyzed. Of the 516 patients enrolled, 464 (89.9%) had started revaccination, with a median time of 2.1 (IQR, 1.8-2.6) years after transplantation and 1.1 (IQR, 0.7-1.5) years after cessation of immunosuppressive therapy. There were 418 cases of hepatitis B surface antibody (HBsAb) seropositive before transplantation, and 339 cases (81.1%) became negative at the time of evaluation after transplantation. The visit time for children with persistently HBsAb seropositive was 1.9 (IQR, 1.3-2.3) years after transplantation, which was shorter than the time for children with HBsAb seronegative (2.2 [IQR, 1.9-2.8] years, P&amp;lt;0.001). HBsAb negative conversion was related to the HBsAb titers pre-transplantation, with a lower proportion of high titers in the negative conversion group (4.7% vs. 17.7%, P&amp;lt;0.001). Both univariate and multivariate analyses suggested that HBsAb seroconversion was statistically related to uncompleted three doses of the hepatitis B vaccine before transplantation, low pre-transplantation antibody titers, and ≥2 years after transplantation. Among 40 patients with HBsAb seronegative who received hepatitis B vaccination, 39 (97.5%) had HBsAb titers converted to seropositive. The immune response rates of measles, mumps, and rubella (MMR) were 96.6% (28/29), 69.0% (20/29,) and 88.5% (23/26), respectively. There were no serious adverse side effects after revaccination.After Allo-HSCT, standard evaluation procedures for revaccination are highly recommended. The immune response rates after hepatitis B and MMR revaccination were highly recommended. No severe adverse side effects were observed.