International Journal of Pediatrics and Adolescent Medicine最新文献

Background and Objective

Despite improvement in medical management, infective endocarditis (IE) remains a serious disease that may affect children with and without preexisting cardiac conditions with significant morbidity and mortality. Neurological complications of IE represent the worst with guarded prognosis. The aim of this study is to describe the incidence, etiology, characteristics, risk factors, and outcome of children with neurological complications associated with IE.

Material and methods

A retrospective cohort study was conducted from 2009 to 2019 where all pediatric patients who fulfilled the modified Duke criteria for IE were included. We divided the cases into 2 groups: IE with neurological complications and IE without neurological complications control group. We compared the two groups statistically and analyzed the results.

Results

We identified 31 (17 male, 14 female) patients with IE. Neurological complications occurred in 7/31 (23%) patients, mainly in the form of a stroke. Gram-positive microbes were the main causative agents for IE (52%) followed by gram-negative (14%), then fungal organisms (3%). Univariate analysis identified the following risk factors for neurological complications: lower body weight, higher C- reactive protein (CRP) level, and left-sided valvular lesions with P values of (0.0003, 0.0001, and 0.04), respectively.

Although mortality was higher in the neurological complications group, it was 43% in comparison to 21% in the control group and it did not reach statistical significance (P = .49). Large vegetation size (more than 10 mm) was seen in 57% of patients with neurological complications as compared to 16% in the control group (P = .052).

Conclusion

Neurological complications occurred in almost a quarter of children with IE. Possible risk factors include lower body weight, left-sided valvular lesion, and higher levels of inflammatory markers (CRP). Stroke was the most common neurological complication encountered with possible increased risk of mortality.

Background

Systemic juvenile idiopathic arthritis (sJIA) is a diagnosis of exclusion. The complex nature and clinical variety of the disease, as well as the vast clinical variation of disease presentation, may lead to difficulties in disease detection and subsequent delays in treatment.

Aim

To provide a consensus guidance on the management of newly diagnosed sJIA patients among pediatric rheumatologists in Arab countries.

Methods

This work was conducted in two phases. The first phase utilized an electronic survey sent through an email invitation to all pediatric rheumatologists in Arab countries. In the second phase, a Task Force of ten expert pediatric rheumatologists from Arab countries met through a series of virtual meetings. Results obtained in phase one were prioritized using a nominal group and Delphi-like techniques in phase two.

Results

Seven overarching principles and a set of recommendations were approved by the Task Force to form the final consensus.

Conclusion

This is the first consensus on a clinical approach for pediatric rheumatic diseases among Arab pediatric rheumatologists. It is presented as a guidance on the clinical approach to sJIA that requires further evidence, and future updates are anticipated.

Background and Objectives

Although child mortality is declining in Saudi Arabia, new trends and causes are emerging. The objective of the study is to determine the causes of child death in a tertiary care hospital in Saudi Arabia and to identify its preventable causes and associated risk factors.

Methods

A modified UNICEF Multiple Indicator Cluster Survey (MICS) was used to analyze all deaths among children under the age of 18 which occurred at the King Abdullah Specialized Children’s Hospital (KASCH) between 2010 and 2016.

Results

After reviewing all the death charts of 1138 children, the team determined that 15% (172) of all deaths could have been prevented and the preventability increased with age. Only 2% of the neonates died of preventable causes, while 53% of the children of 6 years of age or older died of preventable causes. The highest percentage of preventable deaths occurred in children aged 13–18 years (39.3%), followed by the age group of 6–12 years (32.4%) and the age group of 29 days to 5 years (13.9%). All 966 (85%) deaths from biological causes were considered to be unpreventable. Among the preventable causes, 142 (82.5%) had injuries and 30 (17.4%) were sudden unexpected infant death (SUID) with no documented autopsy or death scene investigation, and thus it was considered preventable by the researchers. The 5 major causes of deaths secondary to injuries were motor vehicle accidents (MVA) accounting for 86 deaths (60.6%), followed by drowning accounting for 19 deaths (13.4%), child maltreatment accounting for 13 deaths (9.2%), fire and weapon accounting for 12 deaths (8.5%), and finally home accident (fall, poisoning, suffocation) accounting for 12 deaths (8.5%).

Conclusion

The State Child Death Reviews Board should thoroughly investigate deaths due to SUID and injuries by identifying the factors that contribute to the implementation of preventive strategies.

Background and Aim

According to previously reported studies in the literature, a significant number of patients do not receive enteral nutrition in pediatric intensive care unit (PICU) because of avoidable barriers. Optimal nutrition is a fundamental goal in PICU. This study aims to identify the barriers of enteral nutrition in PICU.

Setting and Design

A cross-sectional study of the results of a 25-item questionnaire-based survey distributed during the Annual International Critical Care Conference by the Saudi Critical Care Society.

Methods and material

A 7-point Likert-type scale was used to rank the participants’ responses, and the relative importance index (RII) approach was used to analyze the relative contribution of each indicator to its main theme.

The factor and parallel analysis methods were used to assess the factorial and unidimensionality of the enteral feeding barriers scale.

Results

A total of 223 PICU healthcare workers from various intensive care settings responded to the survey. The top-three perceived barriers for commencing enteral feeding were due to the patient being hemodynamically unstable (M = 3.6 and SD = 1.70), delays and difficulties in obtaining small bowel access in patients not tolerating other types of enteral nutrition (M = 3.4 and SD = 1.52), or severe fluid restriction, particularly in postoperative cardiac surgery (M = 3.3 and SD = 1.59). The top perceived overall barriers to enteral feeding were the dietician-related issues (M = 3.3, SD = 1.32), barriers related to enteral feeding delivery (M = 3.16 and SD = 1.13), and medical practice-related (M = 3 and SD = 1.10) issues. The lowest reported overall barriers were the resource-related obstacles (M = 2.7 and SD = 1.26).

Conclusion

Being hemodynamically unstable and other dietician-related reasons were the top overall barriers in commencing enteral feeding.

Childhood overgrowth syndromes are relatively rare. A generalized overgrowth syndrome should be suspected when tall stature and macrocephaly are present, after ruling out nutritional excess and endocrinopathies. Sotos syndrome is a well-described overgrowth syndrome due to haploinsufficiency of the NSD1 gene. We present a case of an infant with permanent congenital hypothyroidism, who had tall stature and macrocephaly by 7 months of age. He was noted to have typical facial features, mild gross motor and speech delay, and scoliosis by 13 months of age. Gene sequencing revealed a heterozygous novel c6076_6087del12: p.Asn2026_Thr2029del variant in exon 20 of the NSD1 gene, pathogenic for Sotos syndrome. Congenital hypothyroidism with Sotos syndrome has been infrequently reported and may expand the spectrum of disease characteristics. Early diagnosis of overgrowth syndromes is important for developmental follow up and multidisciplinary care coordination.

Background and Objectives

Child maltreatment (CM) is a major public health problem worldwide. Despite the well-documented cases of all forms of CM and the improvement of the national response to CM in Bahrain, efforts on the prevention of CM are still limited. The objective of this study was to assess the readiness to implement a national evidence-based CM prevention (CMP) program in Bahrain.

Methods

The cross-sectional study was conducted with 45 key informants who had influence and decision-making power over CMP.

Results

The overall score indicated low to moderate readiness. The key informants scored the highest on legislation, mandates, and policies (7.9), which was followed by the knowledge of CM prevention (7.2), institutional resources and links (5.2), and informal social resources (noninstitutional) (5.2). However, the lowest scores were the human and technical resources (1.2), attitudes toward CM prevention (3.3), will to address the problem, and material resources (3.8), current programs implementation and evaluation, and scientific data on CM prevention (4.5).

Conclusion

This research has identified strengths and gaps in the country that needs to be addressed to develop programs that are responsive to the needs of the community. The development of a comprehensive well-resourced CMP program requires the collaborative efforts of legislators, political leaders, and professionals.

Background

Atypical teratoid rhabdoid tumor is an uncommon aggressive central nervous system tumor. All retrospective series have shown a short mean overall survival rate. Considering the rarity of the disease, few prospective clinical trials addressed treatment recommendations for such aggressive tumors, and consequently no definitive treatment guidelines have been established. In this study, we are reviewing our experience in treating atypical teratoid rhabdoid tumor patients.

Methods

We reviewed the medical charts of 43 patients with atypical teratoid rhabdoid tumor who were treated in King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia, between 1996 and 2013. We evaluated the overall survival rate and the influence of different clinical features and treatment protocols on survival.

Results

The median overall survival time was 16.9 months (95% Confidence Interval, 5.2–32.9 months) with an estimated 2- and 5-year overall survival of 41.9% ± 9.6 and 27.9% ± 9.2, respectively. Patients receiving trimodal treatment (surgery, chemotherapy, and radiotherapy) exhibited significantly better median overall survival time compared to their counterparts (P value < .001).

Conclusions

Atypical teratoid rhabdoid tumor is rare and aggressive central nervous system tumor. Despite the limitations of the study, our results support several of clinical practice development. Utilization of postoperative radiotherapy and the adoption of trimodal therapy are associated with significant improvement of median survival. Prompt management with aggressive trimodal therapy should be the standard for future treatment protocols.

Acquired factor VII deficiency secondary to circulating inhibitors is rare in children but is a potentially life-threatening condition. Such a disease is challenging to diagnose and often difficult to manage. Here, we report on a newborn that presented with a catastrophic intracranial hemorrhage who failed to respond to conventional supportive measures including multiple doses of fresh frozen plasma and factor VII replacement; however, he had a complete correction of prothrombin time 8 h after immunomodulatory therapies in the form of steroid and intravenous immunoglobulin. Such measures helped stabilize his bleeding and allowed urgent neurosurgical intervention.

Background/Objective

To draw a hint towards two simple modifications that could potentially decrease the complication rate.

Patients and Methods

It was a single center, single operator and retrospective study. All patients with severe hypospadias operated according to koyanagi with or without modifications were presented. The surgical technique was described. Complications like fistula, stenosis, dehiscence and urethral diverticulum were studied.

Results and Limitations

Nineteen patients were included and presented in a chronological manner. The first four patients were operated according to the original koyanagi technique. The next 15 patients were operated according to koyanagi and the urethroplasty was covered by a tunica vaginalis flap. In 10 of them, glanduloplasty was done primarily and in the last 5 patients, glanduloplasty was done as a secondary procedure. The complication rates were 100% in the original koyanagi group, and 0% in the subset were glanduoplasty was deferred.

Conclusions

Systematically covering the urethroplasty with a tunica vaginalis flap and deferring the glanuloplasty might ameliorate the results of the koyanagi technique.

Background

Gaining vascular access in children is challenging. Ultrasound-guided central line insertion in adults became the standard of care; however, its role in children is not clear. Our objective was to evaluate the ultrasound-guided Port-A-Cath or totally implanted long-term venous access device insertion in pediatric patients compared to the traditional approach.

Methods

This single-institution retrospective cohort study included 169 children who had port-A-catheters between May 2016 and Oct 2019. The patients were divided into two groups; group A included patients who had Port-A-Cath insertion using the landmark method (n = 117), and Group B included patients who had ultrasound-guided Port-A-Cath insertion (n = 52). Preoperative, operative, and postoperative data were collected and compared between the two groups. The study outcomes were operative time and catheter insertion-related complications.

Results

There was no significant difference in age or gender between both groups (P = .33 and .71, respectively). Eleven cases in group A and two cases in group B were converted to cut down technique because of difficulty in inserting the guidewire. There was no difference in the indication of the need for the port-A-Cath between both groups. The mean operative time for group A was 47 min and for group B was 41.7 min (P < .042). Two patients had intraoperative bleeding and hemothorax and required blood transfusion and chest tube insertion in group A. No statistically significant difference was found in the reported complications between the groups. However, the insertion-related complications were higher in group A (P = .053). No procedure-related mortality was reported.

Conclusions

Ultrasound-guided insertion of Port-A-Cath is an effective and safe technique with a reduction of failure rate. It should be considered the standard technique for Port-A-Cath insertion in the pediatric population.