-
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 -
Pain Management Nursing : Official... Jul 2024Morton's neuroma (MN) is one of the most frequent neurological pathologies in feet, affecting approximately 4% of the general population. The treatment of MN can be... (Review)
Review
BACKGROUND
Morton's neuroma (MN) is one of the most frequent neurological pathologies in feet, affecting approximately 4% of the general population. The treatment of MN can be surgical, conservative, and infiltrative, with different substances used in the injections for MN, as steroids, sclerosing solutions, and others. This review aims to evaluate the efficacy of current infiltrative therapy for Morton's neuroma and, additionally, to define adverse effects of this therapy.
MATERIAL AND METHODS
A literature search was performed in PubMed, Embase, CINHAL, Epistemonikos, Web of Science (WOS), SPORTSDiscus and Cochrane Library. This search involved the application of all types of infiltrative treatment applicable to MN. The search was limited to original data describing clinical outcomes and pain using the Visual Analogue pain Scale (VAS) or the Johnson Satisfaction Scale, between February and June 2023.
RESULTS
Twelve manuscripts were selected (six randomized controlled trials and six longitudinal observational studies) involving 1,438 patients. Capsaicin was reported to produce a VAS score reduction of 51.8%. Corticosteroids also reported a high level of efficacy. Alcohol and Hyaluronic Acid injections are well tolerated, but the effects of their application need further research. There were no serious adverse events.
CONCLUSIONS
Corticosteroids, sclerosant injections, hyaluronic acid and capsaicin have been shown to be effective in reducing the pain related to MN.
PubMed: 38955553
DOI: 10.1016/j.pmn.2024.06.005 -
Neurospine Jun 2024Imaging parameters of Chiari malformation type I (CMI) development are not well established. This study aimed to collect evidence of general or specific imaging...
Magnetic Resonance Imaging-Related Anatomic and Functional Parameters for the Diagnosis and Prognosis of Chiari Malformation Type I: A Systematic Review and Meta-analysis.
OBJECTIVE
Imaging parameters of Chiari malformation type I (CMI) development are not well established. This study aimed to collect evidence of general or specific imaging measurements in patients with CMI, analyze indicators that may assist in determining the severity of CMI, and guide its diagnosis and treatment.
METHODS
A comprehensive search was conducted across various databases including the Cochrane Library, PubMed, MEDLINE, Scopus, and Embase, covering the period from January 2002 to October 2023, following predefined inclusion criteria. Meta-analyses were performed using RevMan (ver. 5.4). We performed a quantitative summary and systematic analysis of the included studies. This study was registered in the PROSPERO (International Prospective Register of Systematic Reviews) prior to initiation (CRD42023415454).
RESULTS
Thirty-three studies met our inclusion criteria. The findings indicated that out of the 14 parameters examined, 6 (clivus length, basal angle, Boogard's angle, supraocciput lengths, posterior cranial fossa [PCF] height, and volume) exhibited significant differences between the CMI group and the control group. Furthermore, apart from certain anatomical parameters that hold prognostic value for CMI, functional parameters like tonsillar movement, obex displacement, and cerebrospinal fluid dynamics serve as valuable indicators for guiding the clinical management of the disease.
CONCLUSION
We collated and established a set of linear, angular, and area measurements deemed essential for diagnosing CMI. However, more indicators can only be analyzed descriptively for various reasons, particularly in prognostic prediction. We posit that the systematic assessment of patients' PCF morphology, volume, and other parameters at a 3-dimensional level holds promising clinical application prospects.
PubMed: 38955528
DOI: 10.14245/ns.2347150.575 -
Neurospine Jun 2024
Commentary on "Comparison of the Clinical Efficacy of Anabolic Agents and Bisphosphonates in the Patients With Osteoporotic Vertebral Fracture: Systematic Review and Meta-analysis of Randomized Controlled Trials".
PubMed: 38955519
DOI: 10.14245/ns.2448592.296 -
Ageing Research Reviews Jun 2024Magnolia officinalis, a traditional herbal medicine widely used in clinical practice, exerts antibacterial, anti-tumor, anti-inflammatory, antioxidant, and anti-aging... (Review)
Review
BACKGROUND
Magnolia officinalis, a traditional herbal medicine widely used in clinical practice, exerts antibacterial, anti-tumor, anti-inflammatory, antioxidant, and anti-aging activities. Neolignans are the main active ingredients of M. officinalis and exert a wide range of pharmacological effects, including anti-Alzheimer's disease (AD) activity.
OBJECTIVE
To summarize the published data on the therapeutic effect and mechanism of neolignans on AD in vivo and in vitro.
METHODS
PubMed, Web of Science, Google Scholar, and Scopus were systematically reviewed (up to March 1, 2024) for pre-clinical studies.
RESULTS
M. officinalis-derived neolignans (honokiol, magnolol, 4-O-methylhonokiol, and obovatol) alleviated behavioral abnormalities, including learning and cognitive impairments, in AD animal models. Mechanistically, neolignans inhibited Aβ generation or aggregation, neuroinflammation, and acetylcholinesterase activity; promoted microglial phagocytosis and anti-oxidative stress; alleviated mitochondrial dysfunction and energy metabolism, as well as anti-cholinergic deficiency; and regulated intestinal flora. Furthermore, neolignans may achieve neuroprotection by regulating different molecular pathways, including the NF-κB, ERK, AMPK/mTOR/ULK1, and cAMP/PKA/CREB pathways.
CONCLUSIONS
Neolignans exert anti-AD effects through multiple mechanisms and pathways. However, the exact targets, pharmacokinetics, safety, and clinical efficacy in patients with AD need further investigation in multi-center clinical case-control studies.
PubMed: 38955265
DOI: 10.1016/j.arr.2024.102398 -
Diabetes & Metabolic Syndrome Jun 2024Semaglutide, a glucagon-like peptide-1 receptor agonist, is reported to have cardiac benefits, but its effects on preventing atrial fibrillation (AF) remain...
BACKGROUND
Semaglutide, a glucagon-like peptide-1 receptor agonist, is reported to have cardiac benefits, but its effects on preventing atrial fibrillation (AF) remain inconclusive. This study aimed to investigate whether semaglutide can prevent AF occurrence in patients with type 2 diabetes mellitus (T2DM), obesity, or overweight.
METHODS
We searched MEDLINE, EMBASE, the Cochrane CENTRAL database, and clinicaltrials.gov from inception to December 29, 2023. Randomized controlled trials of semaglutide in patients with T2DM, obesity, or overweight were included. The primary outcome was AF occurrence. Relative risks (RRs) with 95 % confidence intervals (CIs) were calculated for the overall population and subgroups.
RESULTS
Twenty-one trials comprising 25957 patients were included. In the overall pooled analysis, semaglutide decreased AF occurrence compared to control drugs (RR 0.70, 95 % CI 0.52-0.95). This result was consistent in trials using other antihyperglycemic medications as controls (RR 0.43, 95 % CI 0.21-0.89), but not in placebo-controlled trials (RR 0.77, 95 % CI 0.56-1.07). The outcome was favorable for patients with T2DM (RR 0.71, 95 % CI 0.52-0.97), but not for patients with overweight or obesity (RR 0.56, 95 % CI 0.18-1.73). Results varied by type of semaglutide, with oral semaglutide showing an RR of 0.49 (95 % CI 0.25-0.97) and subcutaneous semaglutide showing an RR of 0.77 (95 % CI 0.55-1.07).
CONCLUSION
Semaglutide was associated with a reduced risk of AF occurrence in the overall analysis. Favorable outcomes were observed in subsets using other antihyperglycemic medications as controls, in patients with T2DM, and with oral semaglutide.
PubMed: 38955095
DOI: 10.1016/j.dsx.2024.103067 -
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 -
Seminars in Arthritis and Rheumatism Jun 2024Systemic sclerosis (SSc) is a heterogenous, multi-system autoimmune disease that causes progressive fibrosis of the skin and internal organs, resulting in high morbidity... (Review)
Review
BACKGROUND
Systemic sclerosis (SSc) is a heterogenous, multi-system autoimmune disease that causes progressive fibrosis of the skin and internal organs, resulting in high morbidity and mortality. Intravenous Immunoglobulin (IVIG) is a therapeutic option for SSc; however, reports of its efficacy have been variable, and its use across multiple organ manifestations of SSc has not been comprehensively reviewed.
AIM
The aim of this study was to systematically assess the existing literature on the role of IVIG use across a range of SSc manifestations.
METHODS
Medline, Embase, Cochrane, Web of Science and Scopus were searched from 01/01/2003-15/04/2024 using terms related to SSc and IVIG. Included studies were English-language full texts, where ≥5 adults with SSc received IVIG, and where a reportable outcome was documented.
RESULTS
Of 418 potentially relevant records, 12 were included in this review, comprising 266 patients across one randomised control trial, two pilot studies, one open label study, seven retrospective studies and one case control study. Eighteen outcomes were documented across five different organ systems: cutaneous, respiratory, musculoskeletal, gastrointestinal, and other (clinical improvement and corticosteroid sparing benefit). Results showed a favourable effect of IVIG in reducing the extent of skin thickening, muscle and joint pain, gastrointestinal symptoms, steroid dosing and improving patient/physician reported quality of life. Whilst IVIG may appear to be less beneficial for respiratory disease, the stabilisation in pulmonary function tests and radiological features may be considered a positive outcome in itself. Limitations included a lack of high-quality studies, and the use of concomitant therapies in many studies, rendering the efficacy of IVIG alone difficult to ascertain.
CONCLUSION
IVIG showed benefit in treating some manifestations of SSc, however there was a lack of convincing evidence for the efficacy in others. The lack of high-quality data highlights the need for further well-designed clinical trials to confirm these findings and inform guidelines for IVIG use.
PubMed: 38954999
DOI: 10.1016/j.semarthrit.2024.152471 -
Clinical Neurology and Neurosurgery Jun 2024Subarachnoid hemorrhage (SAH) is associated with high rates of mortality and morbidity, particularly among elderly patients. The presence of frailty may impact survival... (Review)
Review
BACKGROUND
Subarachnoid hemorrhage (SAH) is associated with high rates of mortality and morbidity, particularly among elderly patients. The presence of frailty may impact survival rates in patients with SAH. In this study, we aim to investigate the impact of frailty on the clinical outcomes in SAH patients.
METHODS
We conducted a systematic review and meta-analysis following the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Relevant papers through December 2023 were retrieved from PubMed, Scopus, Web of science, and Embase.
RESULTS
A total of 5 studies met inclusion/exclusion criteria with an aggregate 39,221 non-frail patients (mean age 52.4 ± 5.2 yr; 62.1 % Female), and 79,416 frail patients (mean age 61.1 ± 5.4 yr; 69.0 % Female). Frailty was significantly associated with higher mortality ratio (Odds ratio (OR)= 2.09; CI [1.04: 4.20], p= 0.04), and increased length of hospital stay (OR= 1.40; CI [1.07: 1.83], p= 0.015). Additionally, frailty was associated with higher odds of external ventricular drain insertion, the need of tracheostomy/endoscopic gastrostomy, increased risk of deep vein thrombosis, and postoperative neurological complications.
CONCLUSION
Frailty is associated with worse clinical outcomes and higher mortality rates in SAH patients. Our findings highlight that frailty, when considered alongside other established prognostic factors, serves as crucial predictor for peri-operative complications and overall hospital course in SAH patients.
PubMed: 38954868
DOI: 10.1016/j.clineuro.2024.108413 -
Clinical Neurology and Neurosurgery Jun 2024Pure germinoma typically originates from the midline and is usually found in the pineal and suprasellar regions in 76-90 % of cases. When it is in both regions, it is... (Review)
Review
BACKGROUND
Pure germinoma typically originates from the midline and is usually found in the pineal and suprasellar regions in 76-90 % of cases. When it is in both regions, it is considered bifocal (10 % at diagnosis). If pure germinoma is located outside of the midline, it is considered ectopic, with a global incidence of about 0.7 %. The study aims to describe the clinical and surgical approach to patients with atypical intracranial ectopic germinoma (IEG) and bifocal germinoma (BG) through a literature review with the goal to delineate the correct diagnostic and therapeutic pathway, to reduce the diagnostic delay and improve the prognosis of these patients.
METHODS
A systematic review of the literature in most common electronic database (PubMed, Ovid MEDLINE and Ovid EMBASE) on IEG and BG, in according with the "PRISMA statement" criteria, from January 1990 to September 2022 was done. In addition, two rare cases of IEG and BG were reported.
RESULTS
This systematic review included 16 papers (20 patients) with a final diagnosis of IEG and 30 papers (121 patients) with a final diagnosis of BG. IEGs seems to involve primary basal ganglia (40 %) and corpus callosum (40 %). For IEGs, biopsy (70 %, 14 cases out of 20) was the most common surgical approach: open approach (35 %), stereotactic minimally invasive approach (30 %) or endoscopic trans-sphenoidal approach (5 %). Partial resection was performed in 10 % of cases, whereas a total resection was performed in 20 % of cases. Also for BGs, biopsy was the most common surgical approach in 80 % of patients, whereas surgical resection (partial or total) was performed in 5.3 % of patients.
CONCLUSION
IEG and BG are rare type of primary intracranial germ cell tumor, whose unusual location often can cause delays in diagnosis, which can have a significant impact on the patient's prognosis and requiring a multidisciplinary and timely approach.
PubMed: 38954867
DOI: 10.1016/j.clineuro.2024.108408