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Diabetes Technology & Therapeutics Sep 2023Type 1 diabetes and type 2 diabetes have high rates of associated exocrine pancreatic insufficiency (EPI). This review evaluated the current evidence on prevalence and... (Review)
Review
Type 1 diabetes and type 2 diabetes have high rates of associated exocrine pancreatic insufficiency (EPI). This review evaluated the current evidence on prevalence and treatment of EPI in type 1 and type 2 diabetes and compared general population prevalence rates of EPI and prevalence of other common gastrointestinal conditions such as celiac disease and gastroparesis based on within-diabetes rates of common gastrointestinal (GI) conditions. Prevalence of EPI in type 1 diabetes ranges from 14% to 77.5% (median 33%), while EPI in type 2 diabetes ranges from 16.8% to 49.2% (median 29%), and where type of diabetes is not specified in studies, ranges from 5.4% to 77%. In studies with control groups of the general population, prevalence of EPI overall in those without diabetes ranged from 4.4% to 18%, median 13%, which is comparable with other estimated general population prevalence rates of EPI (10%-20%). Cumulatively, this suggests there may be significant numbers of people with diabetes with EPI who are undiagnosed. People with diabetes (both type 1 and type 2) who present with gastrointestinal symptoms, such as steatorrhea or changes in stool, bloating, and/or abdominal pain, should be screened for EPI. Both diabetes specialists and gastroenterologists and primary care providers should be aware of the high rates of prevalence of diabetes and EPI and recommend fecal elastase-1 screening for people with diabetes and GI symptoms.
Topics: Humans; Diabetes Mellitus, Type 2; Diabetes Mellitus, Type 1; Prevalence; Exocrine Pancreatic Insufficiency; Gastroparesis
PubMed: 37440180
DOI: 10.1089/dia.2023.0157 -
Seizure Aug 2023Dyke-Davidoff-Masson syndrome (DDMS), or cerebral hemiatrophy, was first described in 1933. It is characterised by cerebral injury that causes hypoplasia in one of the... (Review)
Review
INTRODUCTION
Dyke-Davidoff-Masson syndrome (DDMS), or cerebral hemiatrophy, was first described in 1933. It is characterised by cerebral injury that causes hypoplasia in one of the cerebral hemispheres. The disease has different clinical degrees and two aetiologies: congenital and acquired. Radiological findings depend on the degree of injury and the patient's age at the time.
OBJECTIVE
To provide information on the main clinical and radiological characteristics of this disease.
METHODS
A systematic review of the PubMed, MEDLINE, and LILACS databases was conducted using only one keyword. Dyke-Davidoff-Masson syndrome. A total of 223 studies were identified, and the results are presented in tables and graphics.
RESULTS
The mean age of the patients was 19.44 (0-83 years), and the majority were male (55.32%). The most common types of epileptic seizures were generalised tonic-clonic seizures (31 cases), focal impaired awareness seizures (20 cases), focal motor seizures (13 cases), focal to bilateral tonic-clonic seizures (nine cases), and focal myoclonic seizures (one case). The main features of the disease were rapid deep tendon reflexes and extensor cutaneous-plantar tendon reflexes (30 cases - 16%), contralateral hemiparesis or hemiplegia (132 cases - 70%), gait alterations (16 cases - 9%), facial paralysis (nine cases - 5%), facial asymmetry (58 cases - 31%), limb asymmetry (20 cases - 11%), delayed developmental milestones (39 cases - 21%), intellectual disability (87 cases - 46%), and language/speech disorders (29 cases - 15%). Left hemisphere atrophy was the most prevalent.
CONCLUSION
DDMS is a rare syndrome, and several questions regarding this disease remain unanswered. This systematic review aims to elucidate the most common clinical and radiological aspects of the disease and emphasises the need for further investigation.
Topics: Humans; Male; Female; Young Adult; Adult; Middle Aged; Aged; Aged, 80 and over; Magnetic Resonance Imaging; Seizures; Epilepsy; Hemiplegia; Atrophy
PubMed: 37327751
DOI: 10.1016/j.seizure.2023.04.020 -
Current Stem Cell Research & Therapy 2024We designed this systematic review and meta-analysis to estimate the pooled efficacy and safety profile of different types of stem cells in treating patients with... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
We designed this systematic review and meta-analysis to estimate the pooled efficacy and safety profile of different types of stem cells in treating patients with cerebral palsy (CP).
METHODS
We systematically searched PubMed, Scopus, EMBASE, Web of Science, Google Scholar, and also gray literature, including references of the included studies which were published before November 2021. We extracted data regarding the total number of participants, first author, publication year, country of origin, mean age, cell type, cell dose, cell source, method of transplantation, duration of follow-up, Gross motor function, Ashworth scale, and adverse events.
RESULTS
We found 2073 articles by literature search; after deleting duplicates, 1194 remained. Nine articles remained for meta-analysis. The SMD of GMF-66 score (after-before) treatment was 1.5 (95% CI:0.7-2.3) (I = 89.9%, < 0.001). The pooled incidence of (GI) complications after transplantation was 21% (95% CI:9-33%) (I = 56%, P = 0.08). The pooled incidence of fever after transplantation was 18 % (95% CI:6-30%) (I = 87.9%, P = 0.08 < 0.001) Conclusion: The result of this systematic review and meta-analysis show that stem cell therapy in cerebral palsy has neuroprotective properties from anti-inflammatory and anti-apoptotic activities. Stem cell therapy seems to be a promising adjunct to traditional therapies for cerebral palsy patients.
Topics: Humans; Cerebral Palsy; Stem Cell Transplantation
PubMed: 36464870
DOI: 10.2174/1574888X18666221201114756 -
Otolaryngology--head and Neck Surgery :... May 2024Delayed facial nerve palsy (dFNP) secondary to head injury is definitely uncommon. Although the mechanism of immediate facial nerve paralysis is well-studied, its... (Review)
Review
OBJECTIVE
Delayed facial nerve palsy (dFNP) secondary to head injury is definitely uncommon. Although the mechanism of immediate facial nerve paralysis is well-studied, its delayed presentation remains debated. Given the dearth of available information, we reported herein our experience with 2 cases of posttraumatic dFNP. This systematic review aimed to evaluate all available information on dFNP and to assess treatment outcome also comparing conservatively and surgically approaches.
DATA SOURCES
Pubmed, Scopus, and Web of Science databases were systematically screened.
REVIEW METHODS
The protocol of this investigation was registered on PROSPERO in April 2023 and the systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement.
RESULTS
Both patients in the case studies showed a complete recovery within 2 to 3 months after the head trauma. One of them still reported a subjective taste alteration at last control. After the application of the inclusion-exclusion criteria, 9 manuscripts with adequate relevance to this topic were included in the systematic review. The study population consisted of 1971 patients with a diagnosis of posttraumatic facial nerve palsy, of which 128 with a dFNP.
CONCLUSIONS
dFNP due to head trauma is a rarely encountered clinical entity, and optimal treatment still remains to be elucidated. Based on the reported data, it seems rational to propose a conservative approach for dFNP with steroid administration as a first line in most cases, indicating surgery in severe and/or refractory cases.
PubMed: 38769871
DOI: 10.1002/ohn.829 -
BMC Neurology Dec 2023Neuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and...
BACKGROUND
Neuromuscular diseases (NMD) emerged as one of the main side effects of the COVID-19 vaccination. We pooled and summarized the evidence on the clinical features and outcomes of NMD associated with COVID-19 vaccination.
METHODS
We comprehensively searched three databases, Medline, Embase, and Scopus, using the key terms covering "Neuromuscular disease" AND "COVID-19 vaccine", and pooled the individual patient data extracted from the included studies.
RESULTS
A total of 258 NMD cases following COVID-19 have been reported globally, of which 171 cases were Guillain-Barré syndrome (GBS), 40 Parsonage-Turner syndrome (PTS), 22 Myasthenia Gravis (MG), 19 facial nerve palsy (FNP), 5 single fiber neuropathy, and 1 Tolosa-Hunt syndrome. All (100%) SFN patients and 58% of FNP patients were female; in the remaining NMDs, patients were predominantly male, including MG (82%), GBS (63%), and PTS (62.5%). The median time from vaccine to symptom was less than 2 weeks in all groups. Symptoms mainly appeared following the first dose of vector vaccine, but there was no specific pattern for mRNA-based.
CONCLUSION
COVID-19 vaccines might induce some NMDs, mainly in adults. The age distribution and gender characteristics of affected patients may differ based on the NMD type. About two-thirds of the cases probably occur less than 2 weeks after vaccination.
Topics: Adult; Humans; Female; Male; COVID-19 Vaccines; COVID-19; Neuromuscular Diseases; Myasthenia Gravis; Guillain-Barre Syndrome; Bell Palsy; Facial Paralysis
PubMed: 38082244
DOI: 10.1186/s12883-023-03486-y -
The Cochrane Database of Systematic... Aug 2023Despite considerable improvement in outcomes for preterm infants, rates of bronchopulmonary dysplasia (BPD) remain high, affecting an estimated 33% of very low... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Despite considerable improvement in outcomes for preterm infants, rates of bronchopulmonary dysplasia (BPD) remain high, affecting an estimated 33% of very low birthweight infants, with corresponding long-term respiratory and neurosensory issues. Systemic corticosteroids can address the inflammation underlying BPD, but the optimal regimen for prevention of this disease, balancing of the benefits with the potentially meaningful risks of systemic corticosteroids, continues to be a medical quandary. Numerous studies have shown that systemic corticosteroids, particularly dexamethasone and hydrocortisone, effectively treat or prevent BPD. However, concerning short and long-term side effects have been reported and the optimal approach to corticosteroid treatment remains unclear.
OBJECTIVES
To determine whether differences in efficacy and safety exist between high-dose dexamethasone, moderate-dose dexamethasone, low-dose dexamethasone, hydrocortisone, and placebo in the prevention of BPD, death, the composite outcome of death or BPD, and other relevant morbidities, in preterm infants through a network meta-analysis, generating both pairwise comparisons between all treatments and rankings of the treatments.
SEARCH METHODS
We searched the Cochrane Library for all systematic reviews of systemic corticosteroids for the prevention of BPD and searched for completed and ongoing studies in the following databases in January 2023: Cochrane Central Register of Controlled Trials, MEDLINE, Embase, and clinical trial databases.
SELECTION CRITERIA
We included randomized controlled trials (RCTs) in preterm infants (< 37 weeks' gestation) at risk for BPD that evaluated systemic corticosteroids (high-dose [≥ 4 mg/kg cumulative dose] dexamethasone, moderate-dose [≥ 2 to < 4 mg/kg] dexamethasone, low-dose [< 2 mg/kg] dexamethasone, or hydrocortisone) versus control or another systemic corticosteroid.
DATA COLLECTION AND ANALYSIS
Our main information sources were the systematic reviews, with reference to the original manuscript only for data not included in these reviews. Teams of two paired review authors independently performed data extraction, with disagreements resolved by discussion. Data were entered into Review Manager 5 and exported to R software for network meta-analysis (NMA). NMA was performed using a frequentist model with random-effects. Two separate networks were constructed, one for early (< seven days) initiation of treatment and one for late (≥ seven days) treatment initiation, to reflect the different patient populations evaluated. We assessed the certainty of evidence derived from the NMA for our primary outcomes using principles of the GRADE framework modified for application to NMA.
MAIN RESULTS
We included 59 studies, involving 6441 infants, in our analyses. Only six of the included studies provided direct comparisons between any of the treatment (dexamethasone or hydrocortisone) groups, forcing network comparisons between treatments to rely heavily on indirect evidence through comparisons with placebo/no treatment groups. Thirty-one studies evaluated early corticosteroid treatment, 27 evaluated late corticosteroid treatment, and one study evaluated both early and late corticosteroid treatments. Early treatment (prior to seven days after birth): Benefits:NMA for early treatment showed only moderate-dose dexamethasone to decrease the risk of BPD at 36 weeks' postmenstrual age (PMA) compared with control (RR 0.56, 95% CI 0.39 to 0.80; moderate-certainty evidence), although the other dexamethasone dosing regimens may have similar effects compared with control (high-dose dexamethasone, RR 0.71, 95% CI 0.50 to 1.01; low-certainty evidence; low-dose dexamethasone, RR 0.83, 95% CI 0.67 to 1.03; low-certainty evidence). Other early treatment regimens may have little or no effect on the risk of death at 36 weeks' PMA. Only moderate-dose dexamethasone decreased the composite outcome of death or BPD at 36 weeks' PMA compared with control (RR 0.77, 95% CI 0.60 to 0.98; moderate-certainty evidence).
HARMS
Low-dose dexamethasone increased the risk for cerebral palsy (RR 1.92, 95% CI 1.12 to 3.28; moderate-certainty evidence) compared with control. Hydrocortisone may decrease the risk of major neurosensory disability versus low-dose dexamethasone (RR 0.65, 95% CI 0.41 to 1.01; low-certainty evidence). Late treatment (at seven days or later after birth): Benefits: NMA for late treatment showed high-dose dexamethasone to decrease the risk of BPD both versus hydrocortisone (RR 0.66, 95% CI 0.51 to 0.85; low-certainty evidence) and versus control (RR 0.72, CI 0.59 to 0.87; moderate-certainty evidence). The late treatment regimens evaluated may have little or no effect on the risk of death at 36 weeks' PMA. High-dose dexamethasone decreased risk for the composite outcome of death or BPD compared with all other treatments (control, RR 0.69, 95% CI 0.59 to 0.80, high-certainty evidence; hydrocortisone, RR 0.69, 95% CI 0.58 to 0.84, low-certainty evidence; low-dose dexamethasone, RR 0.73, 95% CI 0.60 to 0.88, low-certainty evidence; moderate-dose dexamethasone, RR 0.76, 95% CI 0.62 to 0.93, low-certainty evidence).
HARMS
No effect was observed for the outcomes of major neurosensory disability or cerebral palsy. The evidence for the primary outcomes was of overall low certainty, with notable deductions for imprecision and heterogeneity across the networks.
AUTHORS' CONCLUSIONS
While early treatment with moderate-dose dexamethasone or late treatment with high-dose dexamethasone may lead to the best effects for survival without BPD, the certainty of the evidence is low. There is insufficient evidence to guide this therapy with regard to plausible adverse long-term outcomes. Further RCTs with direct comparisons between systemic corticosteroid treatments are needed to determine the optimal treatment approach, and these studies should be adequately powered to evaluate survival without major neurosensory disability.
Topics: Infant, Newborn; Infant; Humans; Hydrocortisone; Bronchopulmonary Dysplasia; Network Meta-Analysis; Cerebral Palsy; Adrenal Cortex Hormones; Dexamethasone
PubMed: 37650547
DOI: 10.1002/14651858.CD013730.pub2 -
Paediatric Respiratory Reviews Dec 2023Spinal muscular atrophy (SMA) is a severe hereditary lower motor neuron disorder characterised by degeneration of alpha motor neurons in the spinal cord, resulting in... (Review)
Review
Evaluation of the therapeutic efficacy and tolerability of current drug treatments on the clinical outcomes of paediatric spinal muscular atrophy type 1: A systematic review.
Spinal muscular atrophy (SMA) is a severe hereditary lower motor neuron disorder characterised by degeneration of alpha motor neurons in the spinal cord, resulting in progressive weakness and paralysis of proximal muscles. A systematic literature search was carried out by using PRISMA guidelines and searching through different databases that could provide findings of evidence on the health outcomes of the approved therapies for the management of paediatric SMA type 1 regarding efficacy with follow-up in terms of motor and respiratory functions and the tolerability and incidence of adverse drug reactions (ADRs) post-treatment from real-world publications. Half of the publications (50%) had a prospective observational design. Eight studies (66.7%) assessed nusinersen, and three studies (25%) assessed onasemnogene abeparvovec with a duration of follow-up ranging from 6 months to 3 years to evaluate the motor and respiratory functions using different assessment tools, hospitalisation rates, and the tolerability and incidence of ADRs post-treatment. The three currently approved treatments for SMA type 1 provided good support and health outcomes in terms of motor function, respiratory outcomes, reduction of hospitalisations, and improvement of survival. Nevertheless, uncertainties regarding continued improvement after long-term illness and the generalizability of results are still unknown.
Topics: Humans; Child; Spinal Muscular Atrophies of Childhood; Muscular Atrophy, Spinal; Genetic Therapy; Respiration; Observational Studies as Topic
PubMed: 37563072
DOI: 10.1016/j.prrv.2023.06.004 -
Gamification and neurological motor rehabilitation in children and adolescents: a systematic review.Neurologia 2024Gamification consists of the use of games in non-playful contexts. It is widely employed in the motor rehabilitation of neurological diseases, but mainly in adult... (Review)
Review
INTRODUCTION
Gamification consists of the use of games in non-playful contexts. It is widely employed in the motor rehabilitation of neurological diseases, but mainly in adult patients. The objective of this review was to describe the use of gamification in the rehabilitation of children and adolescents with neuromotor impairment.
METHODS
We performed a systematic review of clinical trials published to date on the MEDLINE (PubMed), Scielo, SCOPUS, Dialnet, CINAHL, and PEDro databases, following the PRISMA protocol. The methodological quality of the studies identified was assessed using the PEDro scale.
RESULTS
From a total of 469 studies, 11 clinical trials met the inclusion criteria. We analysed the gamification systems used as part of the rehabilitation treatment of different neuromotor conditions in children and adolescents. Cerebral palsy was the most frequently studied condition (6 studies), followed by developmental coordination disorder (3), neurological gait disorders (1), and neurological impairment of balance and coordination (1).
CONCLUSION
The use of gamification in rehabilitation is helpful in the conventional treatment of neuromotor disorders in children and adolescents, with increased motivation and therapeutic adherence being the benefits with the greatest consensus among authors. While strength, balance, functional status, and coordination also appear to improve, future research should aim to determine an optimal dosage.
Topics: Child; Humans; Adolescent; Gamification; Movement Disorders; Cerebral Palsy; Gait; Neurological Rehabilitation
PubMed: 38065433
DOI: 10.1016/j.nrleng.2023.12.006 -
Orthopedic Research and Reviews 2024Capitellum and trochlea fractures, also referred to as coronal shear fractures of the distal humerus, are infrequent yet challenging intra-articular fractures of the... (Review)
Review
PURPOSE
Capitellum and trochlea fractures, also referred to as coronal shear fractures of the distal humerus, are infrequent yet challenging intra-articular fractures of the elbow. There are a variety of surgical approaches and fixation methods with often variable outcomes. This systematic review investigates interventions, outcomes and complications of capitellum and trochlea fractures.
METHODS
A systematic review of studies published in MEDLINE, EMBASE, Web of Science and Cumulative Index to Nursing and Allied Health literature (CINAHL) was conducted to assess the clinical outcomes of capitellum and trochlea fractures managed surgically. Data on patient demographics, surgical approach, implant usage, postoperative outcomes and complications were compiled.
RESULTS
Forty-one studies met the inclusion criteria with a total of 700 patients. Surgical interventions primarily utilized either the lateral (79%) or antero-lateral (15%) approaches with headless compression screws as the most common fixation method (68%). Clinical outcomes were measured using the Mayo Elbow Performance Index (MEPI) with a mean score of 89.9 (±2.6) and the DASH score with a mean of 16.9 (±7.3). Elbow range of motion showed a mean flexion of 126.3° (±19.4), extension of 5.71° (±11.8), pronation of 75.23° (±12.2), and supination of 76.6° (±9.8). The mean flexion-extension arc was 113.7° (±16.9), and the mean pronation-supination arc was 165.31° (±9.41). Complications occurred in 19.8% of cases, with re-interventions required in 8.3% of cases, mainly due to symptomatic implants and elbow stiffness requiring surgical release. Other complications included implant removal (10.4%), overall reported stiff elbows (6%), nerve palsies (2%), non-union (1.5%), and infection (1.2%).
CONCLUSION
The treatment of capitellum and trochlea fractures yields satisfactory outcomes but has a considerable rate of complications and reoperations primarily due to symptomatic implants and elbow stiffness. There is noteworthy variability in the achieved range of motion, suggesting unpredictable outcomes. Deficits in functionality and range of motion are common after surgery, especially with more complex injury patterns.
PubMed: 38947420
DOI: 10.2147/ORR.S472482 -
Journal of Personalized Medicine Feb 2024Although the reported frequency of diplopia is between 10 to 40% of patients with Parkinson's disease (PD) and other movement disorders, it remains one of the most... (Review)
Review
INTRODUCTION
Although the reported frequency of diplopia is between 10 to 40% of patients with Parkinson's disease (PD) and other movement disorders, it remains one of the most undiagnosed non-motor symptoms. Furthermore, it has a major impact on the quality of life of these patients. The aim of this study is to systematically review the literature regarding the frequency, causes, and implications of diplopia in movement disorders.
METHODOLOGY
An electronic search was conducted in March and June 2023 using the PubMed database in order to identify appropriate studies. Studies that were written in English, that represented observational, analytical studies, and case reports, and that provided information regarding diplopia in movement disorders were included in the systematic review.
RESULTS
A total of 686 articles were identified out of which 43 met the inclusion criteria. The studies included in the systematic review ranged from descriptive studies (case reports and case series) to analytical-observational studies (cross-sectional studies, prospective and retrospective cohort studies, and case-control studies). In Parkinson's disease, the incidence of diplopia ranged from 10 to 38%. In these patients, diplopia was linked to the presence of visual hallucinations and cognitive decline but also to convergence insufficiency and the presence of motor fluctuations. Cases of diplopia secondary to deep brain stimulation were also reported. Diplopia was associated with longer disease duration and worse motor and non-motor scores. Diplopia was also reported in other movement disorders such as multiple system atrophy (frequency as high as 18%) and progressive supranuclear palsy (frequency as high as 39%) and was associated with increased mortality and shorter duration in life span.
CONCLUSIONS
Diplopia occurs in up to 38% of patients with movement disorders and has a negative impact on their health-related quality of life. Treating physicians should actively ask about diplopia and other ophthalmological symptoms, as many patients do not spontaneously report them. The pathophysiology of diplopia is complex, and it involves heterogeneous peripheral and central mechanisms. The management of these patients should involve a multidisciplinary team of health professionals in order to provide appropriate, tailored management.
PubMed: 38541012
DOI: 10.3390/jpm14030270