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The Spine Journal : Official Journal of... Jun 2024Copper deficiency myelopathy (CDM) is a rare disease that can present with spastic quadriparesis and sensory ataxia. As a result, it can precisely mimic cervical... (Review)
Review
BACKGROUND AND CONTEXT
Copper deficiency myelopathy (CDM) is a rare disease that can present with spastic quadriparesis and sensory ataxia. As a result, it can precisely mimic cervical spondylitic myelopathy (CSM). Copper deficiency may be seen following gastric bypass surgery, malabsorption syndromes such as celiac disease, and with excessive exogenous zinc intake. We present a systematic review of the literature for CDM and an illustrative case .
PURPOSE
Provide a systematic review of CDM to highlight the importance of recognizing the consideration of CDM in patients presenting to a spine surgeon with myelopathy that progress despite adequate surgical decompression, or myelopathy concomitant with cytopenia, thus requiring further workup.
STUDY DESIGN/SETTING
Retrospective medical record review and systematic review of the literature PATIENT SAMPLE: PubMed and Ovid-Embase database search was conducted in July 2022 OUTCOME MEASURES: Self-reported measures include PRISMA flow diagram for retrospective review; Physiological measures include retrospective review of MRI imaging of cervical spine; alternate demographic and laboratory value data extracted via literature review METHODS: A PubMed and Ovid-Embase database search was conducted in July 2022 searching for "copper deficiency myelopathy [MeSH]" from 2000 to 2022 via PRISMA guidelines. Following title and abstract review, the following data was extracted from full text: age, sex, etiology, hematological values upon presentation (mean corpuscular volume, white blood count, platelet count, and hemoglobin level), metal serum studies (serum copper, ceruloplasmin, and zinc), 24-hour collection of copper and zinc, and distinct radiographic findings on MRI.
RESULTS
A total of 116 studies were included in this review which contained 198 cases of copper deficiency myelopathy. The mean age was 53.57 ± 14.14 years, with the majority being females (63.8%). The most common etiology was prior gastric surgery (n=55, 36.2 %) followed by excessive zinc consumption from the use of zinc denture cream (n=39, 19.9%)The mean serum copper was 15.67 ± 17.84 (normal=80.0-155.0) mcg/dL and mean ceruloplasmin was 6.43 ± 5.25 (normal=16-45) mg/dL. In spite of appropriate treatment with copper supplementation, only 47 cases (24%) reported improvement in neurological status, and only 10 (5.1%) recovered to baseline. A hyperintense T2 signal abnormality resembling an inverted "v" in the dorsal columns was the most common radiographic abnormality.
CONCLUSION
Pertinent risk factors for copper deficiency myelopathy include prior upper gastrointestinal surgery, zinc excess, and malabsorption. Characteristic laboratory and imaging findings include cytopenia, low serum copper and ceruloplasmin, and distinct inverted "v" T2 signal hyperintensity in the dorsal columns. The neurologic deterioration with copper deficiency will progress in spite of decompressive surgery, and can be devastating and irreversible even with copper supplementation, reinforcing the importance of early detection. We thus recommend patients with myelopathy presenting with a history of gastric bypass, malabsorption syndromes, excessive zinc exposure, cytopenia, or imaging resembling an inverted "v" shaped hyperintense T2 MRI signal in the dorsal columns, should first undergo blood tests for copper, ceruloplasmin, and B12 levels prior to surgical consideration.
PubMed: 38909910
DOI: 10.1016/j.spinee.2024.06.018 -
Patient Preference and Adherence 2024Understanding the quality of life and the factors that influence it for patients with short bowel syndrome (SBS) and their caregivers is of utmost importance in order to... (Review)
Review
PURPOSE
Understanding the quality of life and the factors that influence it for patients with short bowel syndrome (SBS) and their caregivers is of utmost importance in order to enhance their well-being. Therefore, This study aimed to provide a comprehensive understanding of the impact of SBS on patients and their caregivers, as well as its associated factors, by synthesizing the available evidence.
METHODS
A systematic review of the literature was done using PubMed, Embase databases, CNKI, and ISPOR conference papers. Included articles were manually searched to identify any other relevant studies. Quality was assessed using appropriate Joanna Briggs Institute critical appraisal tools.
RESULTS
This review included 16 studies, comprising 15 observational studies and 1 randomized controlled trial. The findings revealed that the QoL of patients with SBS was lower than that of the general population regarding physical functioning and psychological domain. Meanwhile, caregivers experienced challenges in maintaining their QoL. The QoL of SBS patients was found to be influenced by various factors such as treatment, age, sex, stoma, and small intestine length. Among them, the treatment is the most noteworthy factor that can be effectively improved through external interventions.
CONCLUSION
While numerous studies have provided insights into the compromised QoL experienced by individuals with SBS and their caregivers, there remains a scarcity of large-sample quantitative investigations examining the determinants of QoL. The existing body of literature on caregivers is also notably deficient.
PubMed: 38895637
DOI: 10.2147/PPA.S443026 -
The American Journal of Gastroenterology Jun 2024Bile acid sequestrants (BAS) are an option for microscopic colitis (MC) refractory or intolerant to budesonide. There are inconsistent data on the prevalence of bile...
BACKGROUND
Bile acid sequestrants (BAS) are an option for microscopic colitis (MC) refractory or intolerant to budesonide. There are inconsistent data on the prevalence of bile acid malabsorption (BAM) and utility of bile acid testing in MC. The aim of this systematic review and meta-analysis was to evaluate these outcomes.
METHODS
A systematic search of randomized control trials (RCTs) and observational studies of adults with MC treated with BAS was conducted using Medline, Embase, Cochrane, and Scopus from inception to 1/22/2024. Data were extracted on 1) prevalence of BAM, 2) clinical response and adverse events, and 3) recurrence after BAS discontinuation. Data were pooled using random effects models to determine weighted pooled estimates and 95% confidence intervals (CI).
RESULTS
We included 23 studies (1 RCT, 22 observational), with 1,011 MC patients assessed for BAM and 771 treated with BAS. The pooled prevalence of BAM was 34% (95% CI, 0.26-0.42, I2 = 81%). The pooled response rate with BAS induction for all MC patients, irrespective of BAM, was 62% (95% CI, 0.55-0.70, I2 = 71%). There was a higher pooled response rate in patients with BAM compared to those without BAM (p < 0.0001). The pooled rate of BAS-related adverse effects was 9% (95% CI, 0.05-0.14, I2 = 58%).
CONCLUSION
One third of patients with MC had BAM and almost two thirds of all patients responded to BAS with limited side effects. Patients with MC and BAM were more likely to respond to therapy, supporting the value of bile acid testing.
PubMed: 38864509
DOI: 10.14309/ajg.0000000000002886 -
Digestive Diseases (Basel, Switzerland) Jun 2024Celiac disease is an autoimmune condition that affects approximately 1% of the population worldwide. Although its main impact often concerns the small intestine,...
Celiac disease is an autoimmune condition that affects approximately 1% of the population worldwide. Although its main impact often concerns the small intestine, resulting in villous atrophy and nutrient malabsorption, it can also cause systemic manifestations, particularly when undiagnosed or left untreated. Here, attention is paid to the possible psychological, psychiatric, and organic brain manifestations of celiac disease. Specific topics related to the influence and risk of such manifestations with respect to celiac disease are defined and discussed. Overall, eighteen main topics are considered, sifted from over 500 references. The most often studied topics were found to be the effect on quality of life, organic brain dysfunction and ataxia, epilepsy, Down syndrome, generalized psychological disorders, eating dysfunction, depression, and schizophrenia. For most every topic, although many studies report a connection to celiac disease, there are often one or more contrary studies and opinions. A bibliographic analysis of the cited articles was also done. There has been a sharp increase in interest in this research since 1990. Recently published articles tend to receive more referencing, up to as many as 15 citations per year, suggesting an increasing impact of the topics. The number of manuscript pages per article has also tended to increase, up to as many as 12 pages. The impact factor of the publishing journal has remained level over the years. This compendium may be useful in developing a consensus regarding psychological, psychiatric, and organic brain manifestations that can occur in celiac disease, and for determining the best direction for ongoing research focus.
PubMed: 38861947
DOI: 10.1159/000534219 -
Frontiers in Immunology 2024Celiac disease (CD) is an autoimmune chronic enteropathy provoked by gluten ingestion in genetically predisposed individuals. Considering it´s only safe treatment is a... (Review)
Review
Celiac disease (CD) is an autoimmune chronic enteropathy provoked by gluten ingestion in genetically predisposed individuals. Considering it´s only safe treatment is a lifelong gluten-free diet, the burden of living with the disease becomes evident, as well as the need to assess CD health-related quality of life (HRQOL). This review aims to identify and analyze the instruments used to evaluate the HRQOL of adults with CD. This integrative review using a systematic approach was designed to achieve high scientific standards. Accordingly, the search strategy was developed and executed as recommended by the guideline of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Detailed individual searches were developed to Pubmed, Science Direct, Scopus, Web of Science, and Google Scholar. After careful analysis of the papers, 43 studies were included, in which seven instruments were identified: Celiac Disease Questionnaire (CDQ) (n=21), Celiac Disease Specific Quality of Life Instrument (CD-QOL) (n=17), Celiac Disease Assessment Questionnaire (CDAQ) (n=4), CeliacQ-7 (n=1), CeliacQ-27 (n=1), Black and Orfila´s self-developed instrument (n=1) and the Coeliac Disease Quality of Life Questionnaire (CDQL) (n=1). The CDQ and CD-QOL were the two most applied instruments. Since the first focuses on the physical and mental symptoms related to the disease and the second focuses on the emotional repercussions of adhering to the GFD treatment for life (dysphoria), the CDQ application is an interesting option for countries that struggle with public policies for CD patients and patients with active CD. The CD-QOL could be used for countries with strict regulations for CD and gluten-free products and populations in remission. When comparing results among different populations, it is preferable to utilize culturally validated instruments, which have been applied across multiple countries, providing greater comparability between study findings.
Topics: Celiac Disease; Quality of Life; Humans; Surveys and Questionnaires; Diet, Gluten-Free
PubMed: 38742113
DOI: 10.3389/fimmu.2024.1396589 -
Ulusal Travma Ve Acil Cerrahi Dergisi =... May 2024Magnet ingestion in children can lead to serious complications, both acutely and chronically. This case report discusses the treatment approach for a case involving... (Review)
Review
Magnet ingestion in children can lead to serious complications, both acutely and chronically. This case report discusses the treatment approach for a case involving multiple magnet ingestions, which resulted in a jejuno-colonic fistula, segmental intestinal volvulus, hepa-tosteatosis, and renal calculus detected at a late stage. Additionally, we conducted a literature review to explore the characteristics of intestinal fistulas caused by magnet ingestion. A six-year-old girl was admitted to the Pediatric Gastroenterology Department pre-senting with intermittent abdominal pain, vomiting, and diarrhea persisting for two years. Initial differential diagnoses included celiac disease, cystic fibrosis, inflammatory bowel disease, and tuberculosis, yet the etiology remained elusive. The Pediatric Surgery team was consulted after a jejuno-colonic fistula was suspected based on magnetic resonance imaging findings. The physical examination revealed no signs of acute abdomen but showed mild abdominal distension. Subsequent upper gastrointestinal series and contrast enema graphy confirmed a jejuno-colonic fistula and segmental volvulus. The family later reported that the child had swallowed a magnet two years prior, and medical follow-up had stopped after the spontaneous expulsion of the magnets within one to two weeks. Surgical intervention was necessary to correct the volvulus and repair the large jejuno-colonic fistula. To identify relevant studies, we conducted a detailed literature search on magnet ingestion and gastrointestinal fistulas according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. We identified 44 articles encompassing 55 cases where symptoms did not manifest in the acute phase and acute abdomen was not observed. In 29 cases, the time of magnet ingestion was unknown. Among the 26 cases with a known ingestion time, the average duration until fistula detection was 22.8 days (range: 1-90 days). Fistula repairs were performed via laparotomy in 47 cases.
Topics: Humans; Female; Intestinal Fistula; Child; Foreign Bodies; Magnets; Malabsorption Syndromes; Jejunal Diseases; Intestinal Volvulus; Colonic Diseases
PubMed: 38738679
DOI: 10.14744/tjtes.2024.50845 -
Clinical Nutrition (Edinburgh, Scotland) Jun 2024Celiac disease (CD) is a chronic inflammatory disease of the small intestine induced and maintained by gluten ingestion in susceptible individuals. Current treatment... (Review)
Review
BACKGROUND & AIMS
Celiac disease (CD) is a chronic inflammatory disease of the small intestine induced and maintained by gluten ingestion in susceptible individuals. Current treatment consists of strict adherence to a lifelong gluten-free diet (GFD) which is considered safe and effective in the large majority of patients. However, since adherence to a GFD is difficult and has a negative impact on quality of life, an increasing interest in other treatment options has emerged. Moreover, in some individuals a GFD is not sufficiently effective, necessitating alternative treatments.
METHODS
By performing a systematic search, we constructed a detailed narrative review. Only treatment options considered relevant and conducted in a phase I, II or III clinical trial were included.
RESULTS
Based on the pathophysiology of CD, four major therapeutic approaches can be distinguished: firstly, by focusing on intraluminal gluten detoxification before absorption occurs, secondly, by modulating intestinal permeability and preventing paracellular uptake, thirdly, by enhancing immunological tolerance to gluten and finally, by regulating gluten auto-immunity.
CONCLUSIONS
Despite significant efforts, no treatment has yet completed a phase III clinical trial. Future studies will likely focus on the use of supplemental drugs in conjunction to a GFD, with ALV003 and ZED-1227 currently being the most promising therapeutic options.
Topics: Celiac Disease; Humans; Diet, Gluten-Free; Glutens; Peptide Hydrolases
PubMed: 38648685
DOI: 10.1016/j.clnu.2024.04.014 -
Nutrients Apr 2024Persistent symptoms in coeliac disease (CD) can be due to not only poor gluten-free diet (GFD) adherence and complications of CD, but also functional gastrointestinal... (Review)
Review
Persistent symptoms in coeliac disease (CD) can be due to not only poor gluten-free diet (GFD) adherence and complications of CD, but also functional gastrointestinal disorders such as irritable bowel syndrome (IBS). Although the role of a low fermentable oligo-, di-, and monosaccharides and polyols (FODMAP) diet is well-established in IBS, little data are available on its role in coeliac patients with persistent IBS-like symptoms despite a GFD. We systematically reviewed the literature in accordance with the PRISMA guidelines for studies evaluating the role of FODMAPs and/or a low-FODMAP diet in coeliac patients with persistent symptoms. PubMed and Embase were searched from inception to 16 January 2024 for eligible full-text papers. The study protocol was registered on Open Science Framework. A total of 239 records were identified, and six papers were included. Of these, four were interventional studies comparing a low-FODMAP GFD to a regular GFD for persistent symptoms in 115 total coeliac patients (two randomized controlled trials and two open-label studies). A low-FODMAP GFD for a minimum of 4 weeks was significantly more effective than a regular GFD in reducing symptoms ( < 0.05 in 3/4 studies). Dietary FODMAP content of a conventional GFD was significantly lower than that of non-coeliac patients on a gluten-containing diet (both < 0.05), especially regarding high-FODMAP grain products. However, coeliac patients consumed more servings of fruits/vegetables high in FODMAP. No relationship between FODMAP intake and persistence of symptoms was reported. A low-FODMAP diet may be beneficial for uncomplicated celiac patients with persistent IBS-like symptoms despite strict adherence to a GFD.
Topics: Humans; Diet, Gluten-Free; Irritable Bowel Syndrome; FODMAP Diet; Glutens; Celiac Disease
PubMed: 38613127
DOI: 10.3390/nu16071094 -
Clinics and Research in Hepatology and... May 2024Many studies reported the prevalence of extrahepatic conditions (EHC) of primary biliary cholangitis (PBC), but the great heterogeneity existed across different studies.... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND AIM
Many studies reported the prevalence of extrahepatic conditions (EHC) of primary biliary cholangitis (PBC), but the great heterogeneity existed across different studies. Therefore, we conducted the systematic review and meta-analyses to determine EHC prevalence and association with PBC.
METHODS
We searched PUBMED and included observational, cross-sectional and case-controlled studies. A random or fixed effects model was used to estimate the pooled prevalence and odd ratio (OR) as appropriate.
RESULTS
Of 5370 identified publications, 129 publications with 133 studies met the inclusion criteria. Sjögren's syndrome had the highest prevalence (21.4 % vs. 3 % in non-PBC individuals), followed by Raynaud's syndrome (12.3 % vs. 1 %), rheumatoid arthritis-like arthritis (5 % vs. 3 %), systemic sclerosis (3.7 % vs. 0 %) and systemic lupus erythematosus (2 % vs. 0 %). The prevalence of overall thyroid diseases (11.3 %), autoimmune thyroid diseases (9.9 %), osteoporosis (21.1 %), celiac disease (1 %) and chronic bronchitis (4.6 %) was also increased among PBC patients.
CONCLUSION
This is the first exhaustive study on the old theme about EHC of PBC. Given increased prevalence of many EHCs in PBC patients, promptly recognizing these EHCs are of great importance for timely and precise diagnosis of PBC.
Topics: Humans; Prevalence; Liver Cirrhosis, Biliary; Sjogren's Syndrome; Scleroderma, Systemic; Raynaud Disease; Arthritis, Rheumatoid; Celiac Disease; Osteoporosis; Lupus Erythematosus, Systemic; Thyroid Diseases; Autoimmune Diseases
PubMed: 38518985
DOI: 10.1016/j.clinre.2024.102321 -
BMC Pediatrics Mar 2024The relationship between Vitamin D levels and pediatric celiac disease (CD) remains controversial. In this study, we conducted a systematic review and meta-analysis to... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The relationship between Vitamin D levels and pediatric celiac disease (CD) remains controversial. In this study, we conducted a systematic review and meta-analysis to examine the relationship between Vitamin D and pediatric CD.
METHODS
We screened relevant studies from PubMed, EMBASE, and Web of Science published in English from January 1, 2000, to August 1, 2023. The included studies were assessed according to the STROBE checklist. Heterogeneity was quantified by Cochran's Q test and the I statistic. Publication bias was estimated by Begg's test and Egger's test. Meta-regression was used to detect potential sources of heterogeneity.
RESULTS
A total of 26 studies were included in the meta-analysis. Nineteen articles compared 25(OH)D3 levels between CD patients and control groups, average 25-hydroxyvitamin D [25(OH)D or calcidiol], and 1,25-dihydroxyvitamin D [1,25(OH)D or calcitriol] levels, as the main forms of Vitamin D, there was a significant difference in CD patients and healthy controls (weighted mean difference (WMD) = - 5.77, 95% confidence interval (CI) = [- 10.86, - 0.69] nmol/L). Meanwhile, eleven articles reported the numbers of patients and controls with Vitamin D deficiency, there was a significant difference in the incidence of 25(OH)D deficiency between CD patients and healthy controls (odds ratio 2.20, 95% CI= [1.19, 4.08]). Nine articles reported changes in 25(OH)D levels before and after administering a GFD in patients with CD, the result of this study revealed the increase of 25(OH)D levels in CD patients after a gluten-free diet (GFD) (WMD = - 6.74, 95% CI = [- 9.78, - 3.70] nmol/L).
CONCLUSIONS
Vitamin D levels in pediatric CD patients were lower than in healthy controls, and 25(OH)D deficiency was more prevalent in CD patients. We found that 25(OH)D levels were elevated in CD patients after GFD, which is consistent with previous research. Further well-designed, longitudinal, prospective cohort studies focusing on the role of Vitamin D in the pathogenesis of CD are therefore needed.
Topics: Humans; Child; Prospective Studies; Celiac Disease; Vitamin D; Vitamins; Calcitriol; Vitamin D Deficiency
PubMed: 38491474
DOI: 10.1186/s12887-024-04688-0