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Journal of Pediatric Surgery Jun 2024Predicted 1-year survival of children with trisomy 18 (T18) has increased to 59.3%. We aimed to systematically review the characteristics, management, and outcomes of... (Review)
Review
INTRODUCTION
Predicted 1-year survival of children with trisomy 18 (T18) has increased to 59.3%. We aimed to systematically review the characteristics, management, and outcomes of children with T18 and hepatoblastoma.
METHODS
A systematic literature review of the PubMed, Embase, Scopus, Web of Science, and Cochrane Library databases was performed according to the PRISMA 2020 statement (end-of-search date: 03/03/2024).
RESULTS
Fifty studies reporting on 70 patients were included. The median age at diagnosis was 11.5 months, 85.9% were female (n = 55/64), and 15.0% had mosaic T18 (n = 6/40). Diagnosis was made during symptom evaluation (most commonly hepatomegaly or abdominal mass) in 45.5% (n = 15/33), incidentally in 24.2% (n = 8/33), during surveillance with abdominal ultrasound in 18.2% (n = 6/33), and at autopsy in 12.1% (n = 4/33). The median tumor size was 6.4 cm, 33.3% had multiple tumors (n = 14/42), and metastasis was present in one patient (3.8%; n = 1/26). Neoadjuvant chemotherapy was administered in 42.6% (n = 26/61) and adjuvant chemotherapy in 31.6% (n = 18/57). Surgical treatment was performed in 64.2% (n = 43/67). Of the patients not diagnosed on autopsy, overall mortality was 35.5% (n = 22/62) over a median follow-up of 11.0 months. Among the 26 deceased patients (including those diagnosed on autopsy), the most common causes of death were cardiopulmonary disease (38.5%, n = 10/26) and tumor progression (30.8%, n = 8/26).
CONCLUSIONS
T18 does not preclude resection with curative intent for hepatoblastoma. Combination of surgery and chemotherapy should be considered in children on an individualized basis depending on tumor characteristics and underlying cardiopulmonary comorbidities. Locoregional modalities may have a role in the setting of severe comorbidities.
LEVEL OF EVIDENCE
Level IV evidence.
PubMed: 38955626
DOI: 10.1016/j.jpedsurg.2024.06.005 -
Clinical Nutrition (Edinburgh, Scotland) May 2024A dysfunctional hypothalamus may result in decreased feelings of satiety (hyperphagia), decreased energy expenditure, and increased fat storage as a consequence of...
BACKGROUND & AIMS
A dysfunctional hypothalamus may result in decreased feelings of satiety (hyperphagia), decreased energy expenditure, and increased fat storage as a consequence of hyperinsulinemia. Hypothalamic dysfunction may thus lead to morbid obesity and can be encountered in childhood as a consequence of congenital, genetic, or acquired disorders. There is currently no effective treatment for hypothalamic obesity (HO). However, comparable to alimentary obesity, dietary and lifestyle interventions may be considered the cornerstones of obesity treatment. We questioned the effect of dietary or lifestyle interventions for HO and systematically searched the literature for evidence on feasibility, safety, or efficacy of dietary or lifestyle interventions for childhood hypothalamic overweight or obesity.
METHODS
A systematic search was conducted in MEDLINE (including Cochrane Library), EMBASE, and CINAHL (May 2023). Studies assessing feasibility, safety, or efficacy of any dietary or lifestyle intervention in children with hypothalamic overweight or obesity, were included. Animal studies, studies on non-diet interventions, and studies with no full text available were excluded. Because the number of studies to be included was low, the search was repeated for adults with hypothalamic overweight or obesity. Risk of bias was assessed with an adapted Cochrane Risk of Bias Tool. Level of evidence was assessed using the GRADE system. Descriptive data were described, as pooled-data analysis was not possible due to heterogeneity of included studies.
RESULTS
In total, twelve studies were included, with a total number of 118 patients (age 1-19 years) of whom one with craniopharyngioma, one with ROHHAD-NET syndrome, 50 with monogenic obesity, and 66 with Prader-Willi syndrome (PWS). Four studies reported a dietary intervention as feasible. However, parents did experience difficulties with children still stealing food, and especially lowering carbohydrates was considered to be challenging. Seven studies reported on efficacy of a dietary intervention: a well-balanced restrictive caloric diet (30% fat, 45% carbohydrates, and 25% protein) and various hypocaloric diets (8-10 kcal/cm/day) were considered effective in terms of weight stabilization or decrease. No negative effect on linear growth was reported. Four studies reported on specific lifestyle interventions, of which three also included a dietary intervention. Combined dietary and lifestyle intervention resulted in decreased BMI, although BMI returned to baseline values on long-term. One additional study was identified in adults after brain trauma and showed a significant reduction in BMI in one out of eight patients after a combined dietary and lifestyle intervention.
CONCLUSIONS
Hypocaloric diet or restrictive macronutrient diet with lower percentage of carbohydrates seems feasible and effective for childhood HO, although most of the studies had a high risk of bias, small cohorts without control groups, and were conducted in children with PWS only, compromising the generalizability. Lifestyle interventions only resulted in BMI decrease in short-term, indicating that additional guidance is needed to sustain its effect in the long-term. Literature on feasibility and efficacy of a dietary or lifestyle intervention for hypothalamic overweight or obesity is scarce, especially in children with acquired HO (following treatment for a suprasellar tumor). There is need for prospective (controlled) studies to determine which dietary and lifestyle intervention are most helpful for this specific patient group.
PubMed: 38955055
DOI: 10.1016/j.clnu.2024.05.028 -
The Primary Care Companion For CNS... Jun 2024To review the effects of the ketogenic diet on epilepsy in children and adolescents. A literature search was conducted in PubMed with no publication date or language...
To review the effects of the ketogenic diet on epilepsy in children and adolescents. A literature search was conducted in PubMed with no publication date or language restrictions based on the Preferred Reporting Items for Systematic Reviews and Meta Analyses guidelines. Keywords used included , , , , and . After excluding articles that did not meet the inclusion criteria, such as missing variables of study, adult population, and nonrandomized clinical trials, a total of 12 studies were included in the final review. Data on study design, duration, sample size, population, and type of intervention were collected using a standard template. The ketogenic diet and its modified versions were noted to have beneficial effects in reduction of seizure frequency and severity, with manageable adverse effects such as gastrointestinal disturbances, dehydration, dyslipidemia, hyperuricemia, infection, and metabolic acidosis. Depending on patient compliance and comorbidities, all variations of the ketogenic diet were found to be helpful for seizure treatment, whether as an additive or an alternative treatment option, for children and adolescents with epilepsy. .
Topics: Humans; Diet, Ketogenic; Epilepsy; Child; Adolescent
PubMed: 38954792
DOI: 10.4088/PCC.23r03661 -
Clinical Nutrition ESPEN Jun 2024There is a lack of evidence to support the hypothesis that malnutrition may promote cognitive decline. This study aimed to explore the available literature on this topic.
BACKGROUND
There is a lack of evidence to support the hypothesis that malnutrition may promote cognitive decline. This study aimed to explore the available literature on this topic.
METHODS
A systematic review was undertaken of studies investigating the effect of nutritional status on cognitive function in adults of any age, using Medline, Embase, PsycINFO and Global Health via OvidSP from earliest available dates to January 2024. Studies were excluded if they were conducted in animal or paediatric populations, or if they did not include measurements of baseline nutritional status or follow-up assessment of cognitive function. Selected studies were assessed for quality, and data extracted. A meta-analysis was not conducted due to the heterogeneity of the data.
RESULTS
A total of nine studies (three randomised and six observational) was retrieved, including total 8697 subjects who were all in older age groups. Study quality was generally poor. Seven of the nine studies supported the hypothesis that baseline nutritional status is predictive of change in cognitive function at later assessment, but all studies failed to control for significant confounders and six of the nine had large amounts of missing data at follow-up, so that it remains unclear whether nutrition is independently associated with later cognitive function.
CONCLUSION
Malnutrition may be associated with subsequent development of cognitive dysfunction in older adults. Higher quality studies in a wider range of age groups are needed to investigate whether nutritional status has an independent impact on cognitive function, and whether this is related to specific nutrient deficiencies.
PubMed: 38954515
DOI: 10.1016/j.clnesp.2024.06.014 -
Pediatric Surgery International Jul 2024To report our experience with laparoscopic repair of anterior congenital diaphragmatic hernia (CDH) using extracorporeal subcutaneous knot tying and to define recurrence...
PURPOSE
To report our experience with laparoscopic repair of anterior congenital diaphragmatic hernia (CDH) using extracorporeal subcutaneous knot tying and to define recurrence risk factors.
METHODS
This retrospective unicentric study included children who underwent laparoscopic repair of anterior CDH without patch, using extracorporeal knot tying of sutures passed through the full thickness of the abdominal wall (2013-2020). A systematic review of the literature with meta-analysis was performed using the MEDLINE database since 2000.
RESULTS
Eight children were included (12 months [1-183]; 10.6 kg [3.6-65]). Among the two patients with Down syndrome, one with previous cardiac surgery had a recurrence at 17 months postoperatively. In our systematic review (26 articles), among the 156 patients included, 10 had a recurrence (none with patch). Recurrence was statistically more frequent in patients with Down syndrome (19.4%) than without (2.5%) (p < 0.0001), and when absorbable sutures were used (50%) instead of non-absorbable sutures (5.3%) (p < 0.0001).
CONCLUSION
Laparoscopic repair of anterior CDH without patch was a safe and efficient surgical approach in our patients. The use of a non-absorbable prosthetic patch should be specifically discussed in anterior CDH associated with Down syndrome and/or in case of previous cardiac surgery to perform a diaphragmatic tension-free closure.
Topics: Humans; Hernias, Diaphragmatic, Congenital; Laparoscopy; Retrospective Studies; Infant; Recurrence; Herniorrhaphy; Male; Female; Child, Preschool; Child; Suture Techniques; Infant, Newborn; Adolescent; Down Syndrome; Risk Factors
PubMed: 38954216
DOI: 10.1007/s00383-024-05739-4 -
Epilepsia Open Jul 2024To conduct a systematic review of the literature regarding rates and predictors of favorable seizure outcome after resective surgery for epileptic spasms (ES) in... (Review)
Review
To conduct a systematic review of the literature regarding rates and predictors of favorable seizure outcome after resective surgery for epileptic spasms (ES) in pediatric patients. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards were followed. We searched PubMed, EMBASE, and Cochrane CENTRAL for articles published on the prevalence or incidence of epileptic spasm since 1985. Abstract, full-text review, and data extraction were conducted by two independent reviewers. Meta-analysis was performed to assess overall seizure freedom rate. Subject-level analysis was performed on a subset of studies to identify prognostic indicators. A total of 21 retrospective studies (n = 531) were included. Meta-analysis of all studies demonstrated a pooled seizure freedom rate of 68.8%. Subject-level analysis on 18 studies (n = 360) demonstrated a significant association between duration of spasms and recurrence of spasms after surgery, with an estimated increased risk of 7% per additional year of spasms prior to operation. Patients who underwent resective surgery that was not a hemispherectomy (i.e., lobectomy, lesionectomy, etc.) had an increased recurrence risk of 57% compared to patients who had undergone hemispherectomy. Resective surgery results in seizure freedom for the majority of pediatric patients with epileptic spasms. Patients who undergo hemispherectomy have lower risk of recurrence than patients who undergo other types of surgical resection. Increased duration of spasms prior to surgery is associated with increased recurrence risk after surgery. PLAIN LANGUAGE SUMMARY: Children with epileptic spasms (ES) that do not respond to medications may benefit from surgical treatment. Our study reviewed existing research to understand how effective surgery is in treating ES in children and what factors predict better outcomes. Researchers followed strict guidelines to search for and analyze studies published since 1985, finding 21 studies with a total of 531 patients. They found that, on average, nearly 70% of children became seizure-free after surgery. Further individual analysis of 360 patients showed that longer duration of spasms before surgery increased the risk of spasms returning by 7% per year. Additionally, children who had less extensive surgeries, such as removal of only a specific part of the brain, had a 57% higher risk of seizure recurrence compared to those who had a hemispherectomy, which removed or disconnected half of the brain. Overall, the study concludes that surgery can often stop seizures, especially when more extensive surgery is performed and when the surgery is done sooner rather than later.
PubMed: 38953892
DOI: 10.1002/epi4.13007 -
Pediatric Pulmonology Jul 2024Several techniques can be used to assess bronchodilator response (BDR) in preschool-aged children, including spirometry, respiratory oscillometry, the interrupter... (Review)
Review
BACKGROUND
Several techniques can be used to assess bronchodilator response (BDR) in preschool-aged children, including spirometry, respiratory oscillometry, the interrupter technique, and specific airway resistance. However, there has not been a systematic comparison of BDR thresholds across studies yet.
METHODS
A systematic review was performed on all studies up to May 2023 measuring a bronchodilator effect in children 2-6 years old using one of these techniques (PROSPERO CRD42021264659). Studies were identified using MEDLINE, Cochrane, EMBASE, CINAHL via EBSCO, Web of Science databases, and reference lists of relevant manuscripts.
RESULTS
Of 1224 screened studies, 43 were included. Over 85% were from predominantly European ancestry populations, and only 22 studies (51.2%) calculated a BDR cutoff based on a healthy control group. Five studies included triplicate testing with a placebo to account for the within-subject intrasession repeatability. A relative BDR was most consistently reported by the included studies (95%) but varied widely across all techniques. Various statistical methods were used to define a BDR, with six studies using receiver operating characteristic analyses to measure the discriminative power to distinguish healthy from wheezy and asthmatic children.
CONCLUSION
A BDR in 2- to 6-year-olds cannot be universally defined based on the reviewed literature due to inconsistent methodology and cutoff calculations. Further studies incorporating robust methods using either distribution-based or clinical anchor-based approaches to define BDR are required.
PubMed: 38953717
DOI: 10.1002/ppul.27112 -
European Heart Journal. Cardiovascular... Jul 2024There is increasing evidence that plaque instability in the extracranial carotid artery may lead to an increased stroke risk independently of the degree of stenosis. We...
AIMS
There is increasing evidence that plaque instability in the extracranial carotid artery may lead to an increased stroke risk independently of the degree of stenosis. We aimed to determine diagnostic accuracy of vulnerable and stable plaque using noninvasive imaging modalities when compared to histology in patients with symptomatic and asymptomatic carotid atherosclerosis.
METHODS AND RESULTS
Medline Ovid, Embase, Cochrane Library, and Web of Science were searched for diagnostic accuracy of noninvasive imaging modalities (CT, MRI, US) in the detection of 1) vulnerable/stable plaque, and 2) vulnerable/stable plaque characteristics, compared to histology. The quality of included studies was assessed by QUADAS-2 and univariate and bivariate random-effect meta-analyses were performed. We included 36 vulnerable and 5 stable plaque studies in the meta-analysis, and out of 211 plaque characteristics from remaining studies, we classified 169 as vulnerable and 42 as stable characteristics (28 CT, 120 MRI, 104 US characteristics). We found that MRI had high accuracy [90% (95% CI: 82-95%)] in the detection of vulnerable plaque, similar to CT [86% (95% CI: 76-92%); P > 0.05], whereas US showed less accuracy [80% (95% CI: 75-84%); P = 0.013]. CT showed high diagnostic accuracy in visualizing characteristics of vulnerable or stable plaques (89% and 90%) similar to MRI (86% and 89%; P > 0.05); however, US had lower accuracy (77%, P < 0.001 and 82%, P > 0.05).
CONCLUSION
CT and MRI have a similar, high performance in detecting vulnerable carotid plaques, whereas US showed significantly less diagnostic accuracy. Moreover, MRI visualized all vulnerable plaque characteristics allowing for a better stroke risk assessment.
REGISTRATION
PROSPERO ID CRD42022329690.
PubMed: 38953552
DOI: 10.1093/ehjci/jeae144 -
The Journal of Asthma : Official... Jul 2024This systematic review aimed to investigate the epidemiological data about meteorological factors and climate change (CC) impact on asthma. A search was performed using... (Review)
Review
This systematic review aimed to investigate the epidemiological data about meteorological factors and climate change (CC) impact on asthma. A search was performed using three databases (Web of Science, Science Direct, and MEDLINE) for all relevant studies published from January 1, 2018, to December 31, 2022. This systematic review complied with the PRISMA document's requirements, including studies related to meteorological factors and CC impact on asthma. The search included studies published in English or French language, and was based on title, abstract, and complete text. Documents not meeting inclusion requirements were excluded. We identified 18 studies published in the last five years that were eligible for inclusion in this review. We found that these studies concerned European, Asian, American, and Oceanic cities. Extreme variations in temperature, humidity, wind speed, exceptional incidents like hurricanes, cold and heat waves, and seasonal shifts were strongly correlated with the worsening of asthmatic symptoms, particularly in childhood. In addition, excessive concentrations of air pollutants and aeroallergens were linked to pediatric asthma emergency hospital admissions. A significant association between the consequences of CC and asthma in adults particularly in children has been demonstrated. Future research should quantify the impact of global change in climate regarding the aeroallergens' distribution in terms of geography and time. It is also necessary to research the impact of air pollution on asthmatic health, like sulfur dioxide (SO), nitrogen dioxide (NO), ozone (O), and particles having an aerodynamic diameter lower than 2.5 µm (PM).
PubMed: 38953539
DOI: 10.1080/02770903.2024.2375272 -
Otolaryngology--head and Neck Surgery :... Jul 2024Arnold-Chiari Malformation is one possible cause of congenital vocal cord paralysis (VCP). The natural history of VCP in children with Chiari malformation has previously... (Review)
Review
OBJECTIVE
Arnold-Chiari Malformation is one possible cause of congenital vocal cord paralysis (VCP). The natural history of VCP in children with Chiari malformation has previously been limited to small case studies. This systematic review seeks to better characterize the prognostic factors that may predict symptom severity and resolution of congenital VCP in children with Arnold-Chiari malformation. We hypothesized that the onset of stridor or VCP at a younger age would be associated with a poorer prognosis and earlier intervention with posterior fossa decompression would be associated with better outcomes.
DATA SOURCES
PubMed, Web of Science, Cochrane Library, and bibliographic review.
REVIEW METHODS
A systematic review was performed according to Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Database search yielded 866 articles. Study abstracts were reviewed by 2 independent examiners. One hundred and seventy-six studies underwent full-text review. The following were extracted: age at onset of stridor or VCP, Chiari malformation type, laryngoscopy findings, type and timing of neurosurgical intervention, and tracheostomy history. Statistical analyses utilized χ tests.
RESULTS
Younger age at symptom onset showed statistically significant associations with decreased likelihood for symptom resolution and tracheostomy decannulation. The shorter time interval from symptom onset to neurosurgical intervention was not significantly associated with better outcomes.
CONCLUSION
This meta-analysis suggests poorer prognosis in those with earlier-onset symptoms, reinforcing prior case series findings. Additional prospective studies are needed to elucidate the natural history and utility of early intervention in children with vocal cord paralysis secondary to Chiari malformation.
PubMed: 38953205
DOI: 10.1002/ohn.884