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Journal of Pediatric Gastroenterology... Apr 2013International trends in incidence and outcomes of biliary atresia (BA) are controversial and a wide range of estimates have been reported worldwide. We reviewed the... (Review)
Review
OBJECTIVES
International trends in incidence and outcomes of biliary atresia (BA) are controversial and a wide range of estimates have been reported worldwide. We reviewed the population-based literature to assess international variation of BA incidence and outcomes, and to assess the evidence for seasonal variation in incidence, centralization of Kasai hepatoportoenterostomy, and newborn screening.
METHODS
We conducted a systematic review (registration number CRD42011001441) of observational or interventional research within MEDLINE, EMBASE, and the Cochrane Database, which reported incidence, prevalence, or outcomes of infants with BA. Population-based studies, defined by inclusion of an entire population or representative sample, were included. Outcomes included overall survival, native liver survival (NLS), and time to Kasai hepatoportoenterostomy. Single- or multicenter studies were excluded unless those centers captured all potential patients within a jurisdiction. Two independent data extractors reviewed the abstracts and articles.
RESULTS
A total of 40 studies were included following review of 3128 references. A wide range of incidence was reported internationally. Ten-year overall survival ranged from 66.7% to 89%. NLS ranged from 20.3% to 75.8% at 1 to 3 years and 24% to 52.8% at 10 years. Earlier age at Kasai was a predictor of improved NLS. Seasonality was reported in 11 studies, and 3 reported an increased incidence during the months of August to March. The evidence for centralization of Kasai to high-volume centers is promising but does not account for all case-mix, provider, or health system factors involved in volume-outcome relations. Stool color card screening resulted in earlier Kasai and improved NLS in Taiwan.
CONCLUSIONS
Large, international studies could help fill the gaps in knowledge identified by this review.
Topics: Biliary Atresia; Child; Global Health; Humans; Incidence; Prognosis; Referral and Consultation; Seasons; Survival Analysis
PubMed: 23263590
DOI: 10.1097/MPG.0b013e318282a913 -
Journal of Thoracic Disease May 2017The characteristics of patients with urinothorax (UT) are poorly defined.
BACKGROUND
The characteristics of patients with urinothorax (UT) are poorly defined.
METHODS
A systematic review was performed searching for studies reporting clinical findings, pleural fluid (PF) characteristics, and the most effective treatment of UT. Case descriptions and retrospective studies were included.
RESULTS
The review included 78 studies with a total of 88 patients. Median age was 45 years, male/female ratio was 1.6:1 and in 76% of cases the etiology was trauma. Pleural effusion (PE) was predominantly unilateral (87%) and occupied over 2/3 of the hemithorax in most cases (64.4%). PF was straw-colored (72.7%) or hematic (27.3%) with urine-like odor in all cases. PF was transudate in 56.2% of cases (18/32) and among 14 exudates (43.8%), 3 were concordant exudates, 1 protein-discordant and 10 LDH-discordant, with lymphocyte (44.4%) and neutrophil (38.5%) predominance. The PF/serum (PF/S) creatinine ratio was >1 in all cases except one (97.9%). The diagnosis was established on the basis of PF/S creatinine ratio >1 (56.6%), urinary tract contrast extravasation (12%), abnormal computed tomography (8.4%), laparotomy findings (6%), and association of obstructive uropathy with PE (6%). The outcome was favorable (74/77; 96.1%) when treatment was direct towards the uropathy (alone or associated with thoracentesis/thoracic drainage). Outcome was unfavorable in the 15 patients who were only treated with thoracentesis/thoracic drainage.
CONCLUSIONS
UT is usually traumatic, unilateral, and PF does not have a specific pattern or cellularity predominance, with a PF/S creatinine ratio almost always >1. Treatment should include the uropathy, with or without PF evacuation.
PubMed: 28616270
DOI: 10.21037/jtd.2017.04.22 -
Surgical Endoscopy Sep 2014Surgeons have attempted to minimize postoperative anastomotic complications by employing intraoperative tests and manoeuvres to assess colorectal anastomotic integrity.... (Review)
Review
BACKGROUND
Surgeons have attempted to minimize postoperative anastomotic complications by employing intraoperative tests and manoeuvres to assess colorectal anastomotic integrity. These have evolved over time with improvement in operative technology and techniques. This systematic review aims to examine the impact of such intraoperative assessments.
METHODS
A systematic review of studies assessing intraoperative anastomotic assessments and their impact on postoperative anastomotic complications was performed. Intraoperative measures undertaken as a result of intraoperative assessments and postoperative anastomotic complications were analysed.
RESULTS
37 Studies were identified. 13 studies evaluated basic mechanical patency tests, ten studies evaluated endoscopic visualisation techniques and 14 studies evaluated microperfusion techniques. Postoperative anastomotic complications were significantly lower in patients tested with basic mechanical patency tests compared to those untested (non-RCT: 4.1 vs. 8.1 %, p = 0.03, RCTs: 5.8 vs. 16.0 %, p = 0.024). There were no differences in postoperative anastomotic complications between tested and non-tested cohorts in non-randomised cohort studies evaluating endoscopic visualisation techniques. However, intraoperative measures taken after abnormal intraoperative tests may have reduced the number of postoperative complications. Perfusion analysis techniques are not in routine widespread clinical practice as yet, but newer techniques such as fluorescent dyes and imaging under near infrared light show technical feasibility.
CONCLUSIONS
Intraoperative colorectal anastomotic assessment has evolved together with advancement of technology in the surgical setting. Moderate benefit in terms of lower postoperative anastomotic complications has been shown with basic mechanical patency testing and more recently with intraoperative endoscopic visualisation of colorectal anastomoses. The next advance and possible introduction into routine practice may include the use of microperfusion techniques. The latest in this group of techniques, which utilise autofluorescent dyes such as Indocyanine green, hold great potential. Well-planned controlled studies or ideally, randomised controlled trials need to be conducted to further assess the benefit of these latest techniques.
Topics: Anastomosis, Surgical; Colon; Coloring Agents; Humans; Indocyanine Green; Monitoring, Intraoperative; Postoperative Complications; Rectum
PubMed: 24718665
DOI: 10.1007/s00464-014-3520-z -
Ophthalmology May 2024To develop guidelines for ocular surveillance and early intervention for individuals with von Hippel-Lindau (VHL) disease.
PURPOSE
To develop guidelines for ocular surveillance and early intervention for individuals with von Hippel-Lindau (VHL) disease.
DESIGN
Systematic review of the literature.
PARTICIPANTS
Expert panel of retina specialists and ocular oncologists.
METHODS
A consortium of experts on clinical management of all-organ aspects of VHL disease was convened. Working groups with expertise in organ-specific features of VHL disease were tasked with development of evidence-based guidelines for each organ system. The ophthalmology subcommittee formulated questions for consideration and performed a systematic literature review. Evidence was graded for topic quality and relevance and the strength of each recommendation, and guideline recommendations were developed.
RESULTS
The quality of evidence was limited, and no controlled clinical trial data were available. Consensus guidelines included: (1) individuals with known or suspected VHL disease should undergo periodic ocular screening (evidence type, III; evidence strength, C; degree of consensus, 2A); (2) patients at risk of VHL disease, including first-degree relatives of patients with known VHL disease, or any patient with single or multifocal retinal hemangioblastomas (RHs), should undergo genetic testing for pathologic VHL disease gene variants as part of an appropriate medical evaluation (III/C/2A); (3) ocular screening should begin within 12 months after birth and continue throughout life (III/C/2A); (4) ocular screening should occur approximately every 6 to 12 months until 30 years of age and then at least yearly thereafter (III/C-D/2A); (5) ocular screening should be performed before a planned pregnancy and every 6 to 12 months during pregnancy (IV/D/2A); (6) ultra-widefield color fundus photography may be helpful in certain circumstances to monitor RHs, and ultra-widefield fluorescein angiography may be helpful in certain circumstances to detect small RHs (IV/D/2A); (7) patients should be managed, whenever possible, by those with subspecialty training, with experience with VHL disease or RHs, or with both and ideally within the context of a multidisciplinary center capable of providing multiorgan surveillance and access to genetic testing (IV/D/2A); (8) extramacular or extrapapillary RHs should be treated promptly (III/C/2A).
CONCLUSIONS
Based on available evidence from observational studies, broad agreement was reached for a strategy of lifelong surveillance and early treatment for ocular VHL disease. These guidelines were endorsed by the VHL Alliance and the International Society of Ocular Oncology and were approved by the American Academy of Ophthalmology Board of Trustees.
FINANCIAL DISCLOSURE(S)
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
Topics: Humans; Fluorescein Angiography; Genetic Testing; Hemangioblastoma; Retina; Retinal Neoplasms; von Hippel-Lindau Disease; Von Hippel-Lindau Tumor Suppressor Protein
PubMed: 38092079
DOI: 10.1016/j.ophtha.2023.12.014 -
Cardiovascular and Interventional... Aug 2018Sclerotherapy has become an important treatment option in the management of vascular malformations. However, little is known about success rate for treatment of venous... (Review)
Review
OBJECTIVE
Sclerotherapy has become an important treatment option in the management of vascular malformations. However, little is known about success rate for treatment of venous malformations. This systematic review assesses the available published literature on outcome measures of sclerotherapy for venous malformations.
DATA SOURCE
PubMed and EMBASE.
REVIEW METHOD
A systematic search was conducted, and studies from March 2008 to October 2016 were included. Based on the identified search results, study selection, data extraction, and assessment of study quality were, according to PRISMA, undertaken independently by two reviewers.
RESULTS
One randomized controlled trial and 44 cohort studies were included. The most frequently used measuring methods were subjective measurements, objective measurements, imaging like MRI and ultrasound, and finally patient reported outcome measure survey. Approximately 60% of the studies used more than one measuring method. Outcome measures were pain, swelling, disfigurement, cosmetic complains, patient satisfaction, physical difficulties, volume, diameter, mass, color, shape, size, venous flow, occlusion of venous space, blood pool ratio, and radioisotope uptake.
CONCLUSIONS
The published literature over the past 10 years shows no clear method to evaluate the effect of sclerotherapy for venous malformation. We suggest that a standard set of outcome measures should be defined.
Topics: Adult; Female; Humans; Male; Patient Satisfaction; Sclerotherapy; Treatment Outcome; Vascular Malformations
PubMed: 29492631
DOI: 10.1007/s00270-018-1919-y -
Genes, Chromosomes & Cancer Mar 2003Sézary syndrome (SS) is a rare form of erythrodermic cutaneous T-cell lymphoma with hematological involvement and a poor prognosis. At present little is known about the... (Comparative Study)
Comparative Study Review
Sézary syndrome (SS) is a rare form of erythrodermic cutaneous T-cell lymphoma with hematological involvement and a poor prognosis. At present little is known about the molecular pathogenesis of this malignancy. To address this issue, we analyzed 28 SS cases through the use of molecular cytogenetic techniques. Conventional cytogenetic analysis showed 12 of 28 cases with clonal chromosome abnormalities (43%). Seven cases had aberrations affecting chromosomes 1 and 17; five demonstrated rearrangement of chromosomes 10 and 14; four presented with an abnormality of 6q. Multiplex-fluorescence in situ hybridization (M-FISH) revealed complex karyotypes in 6 of 17 cases (35%), and recurrent der(1)t(1;10)(p2;q2) and der(14)t(14;15)(q;q?) translocations were each identified in two cases, and confirmed by dual-color FISH. There was an overall difference in the incidence of clonal abnormalities detected by G-banded karyotyping and M-FISH. In addition, comparative genomic hybridization studies revealed chromosome imbalances (CIs) in 9 of 20 cases (45%), with a mean DNA copy number change per sample of 1.95 +/- 2.74, and losses (mean: 1.25 +/- 1.77) more frequent than gains (mean: 0.7 +/- 1.26). The most common CIs noted were loss of 1p, followed by losses of 10/10q, 17p, and 19, and gains of 17q and 18. Furthermore, in conjunction with this study a systematic literature review was conducted, which showed a high frequency and consistent pattern of chromosome changes in SS. These findings suggest that chromosomal instability is common in SS, although there are specific chromosomal abnormalities that appear to be characteristic, and the identification of two different recurrent chromosome translocations provides the basis for further studies.
Topics: Chromosome Aberrations; Chromosome Banding; Chromosome Deletion; Chromosome Painting; Cytogenetic Analysis; Female; Gene Amplification; Humans; In Situ Hybridization, Fluorescence; Male; Nucleic Acid Hybridization; Sezary Syndrome; Translocation, Genetic
PubMed: 12557225
DOI: 10.1002/gcc.10152 -
Health Technology Assessment... Dec 2014Age-related macular degeneration is the most common cause of sight impairment in the UK. In neovascular age-related macular degeneration (nAMD), vision worsens rapidly... (Review)
Review
Optical coherence tomography for the diagnosis, monitoring and guiding of treatment for neovascular age-related macular degeneration: a systematic review and economic evaluation.
BACKGROUND
Age-related macular degeneration is the most common cause of sight impairment in the UK. In neovascular age-related macular degeneration (nAMD), vision worsens rapidly (over weeks) due to abnormal blood vessels developing that leak fluid and blood at the macula.
OBJECTIVES
To determine the optimal role of optical coherence tomography (OCT) in diagnosing people newly presenting with suspected nAMD and monitoring those previously diagnosed with the disease.
DATA SOURCES
Databases searched: MEDLINE (1946 to March 2013), MEDLINE In-Process & Other Non-Indexed Citations (March 2013), EMBASE (1988 to March 2013), Biosciences Information Service (1995 to March 2013), Science Citation Index (1995 to March 2013), The Cochrane Library (Issue 2 2013), Database of Abstracts of Reviews of Effects (inception to March 2013), Medion (inception to March 2013), Health Technology Assessment database (inception to March 2013).
REVIEW METHODS
Types of studies: direct/indirect studies reporting diagnostic outcomes.
INDEX TEST
time domain optical coherence tomography (TD-OCT) or spectral domain optical coherence tomography (SD-OCT).
COMPARATORS
clinical evaluation, visual acuity, Amsler grid, colour fundus photographs, infrared reflectance, red-free images/blue reflectance, fundus autofluorescence imaging, indocyanine green angiography, preferential hyperacuity perimetry, microperimetry. Reference standard: fundus fluorescein angiography (FFA). Risk of bias was assessed using quality assessment of diagnostic accuracy studies, version 2. Meta-analysis models were fitted using hierarchical summary receiver operating characteristic curves. A Markov model was developed (65-year-old cohort, nAMD prevalence 70%), with nine strategies for diagnosis and/or monitoring, and cost-utility analysis conducted. NHS and Personal Social Services perspective was adopted. Costs (2011/12 prices) and quality-adjusted life-years (QALYs) were discounted (3.5%). Deterministic and probabilistic sensitivity analyses were performed.
RESULTS
In pooled estimates of diagnostic studies (all TD-OCT), sensitivity and specificity [95% confidence interval (CI)] was 88% (46% to 98%) and 78% (64% to 88%) respectively. For monitoring, the pooled sensitivity and specificity (95% CI) was 85% (72% to 93%) and 48% (30% to 67%) respectively. The FFA for diagnosis and nurse-technician-led monitoring strategy had the lowest cost (£ 39,769; QALYs 10.473) and dominated all others except FFA for diagnosis and ophthalmologist-led monitoring (£ 44,649; QALYs 10.575; incremental cost-effectiveness ratio £ 47,768). The least costly strategy had a 46.4% probability of being cost-effective at £ 30,000 willingness-to-pay threshold.
LIMITATIONS
Very few studies provided sufficient information for inclusion in meta-analyses. Only a few studies reported other tests; for some tests no studies were identified. The modelling was hampered by a lack of data on the diagnostic accuracy of strategies involving several tests.
CONCLUSIONS
Based on a small body of evidence of variable quality, OCT had high sensitivity and moderate specificity for diagnosis, and relatively high sensitivity but low specificity for monitoring. Strategies involving OCT alone for diagnosis and/or monitoring were unlikely to be cost-effective. Further research is required on (i) the performance of SD-OCT compared with FFA, especially for monitoring but also for diagnosis; (ii) the performance of strategies involving combinations/sequences of tests, for diagnosis and monitoring; (iii) the likelihood of active and inactive nAMD becoming inactive or active respectively; and (iv) assessment of treatment-associated utility weights (e.g. decrements), through a preference-based study.
STUDY REGISTRATION
This study is registered as PROSPERO CRD42012001930.
FUNDING
The National Institute for Health Research Health Technology Assessment programme.
Topics: Cost-Benefit Analysis; Fluorescein Angiography; Humans; Macular Degeneration; Models, Econometric; Quality-Adjusted Life Years; Tomography, Optical Coherence; Visual Acuity
PubMed: 25436855
DOI: 10.3310/hta18690 -
International Journal of Environmental... Feb 2022Regional odontodysplasia is a rare developmental disorder characterised by hypoplasia and hypomineralisation of enamel and dentin. Our systematic review aimed to... (Review)
Review
Regional odontodysplasia is a rare developmental disorder characterised by hypoplasia and hypomineralisation of enamel and dentin. Our systematic review aimed to organise the knowledge on localisation, symptomatology and treatment methods in patients with regional odontodysplasia based on case reports published in the databases PubMed, Scopus and Web of Science. Case reports were described in 28 different countries, considering 180 patients (including 91 females). Regional odontodysplasia occurs mainly in both deciduous and permanent dentition (66.1%). The affected teeth were observed more frequently in the maxilla (70.0%), especially on the left side (45.6%). The most common reported symptoms were ghost teeth, poorly developed buds, yellowish-brown colour of crowns and delayed eruption of permanent teeth in affected quadrants. The most popular treatment method was surgical treatment (78.6%) with subsequent prosthetic therapy (34.6%). Based on the review of cases, pathognomonic clinical and radiological signs can be found, however, it is difficult to reach a consensus on the choice of treatment method.
Topics: Bibliometrics; Dentition, Permanent; Female; Humans; Maxilla; Odontodysplasia; Radiography; Tooth, Deciduous
PubMed: 35162705
DOI: 10.3390/ijerph19031683 -
Genetics in Medicine : Official Journal... Mar 2022This study aimed to systematically review and summarize gene therapy treatment for monogenic retinal and optic nerve diseases. (Review)
Review
PURPOSE
This study aimed to systematically review and summarize gene therapy treatment for monogenic retinal and optic nerve diseases.
METHODS
This review was prospectively registered (CRD42021229812). A comprehensive literature search was performed in Ovid MEDLINE, Ovid Embase, Cochrane Central, and clinical trial registries (February 2021). Clinical studies describing DNA-based gene therapy treatments for monogenic posterior ocular diseases were eligible for inclusion. Risk of bias evaluation was performed. Data synthesis was undertaken applying Synthesis Without Meta-analysis guidelines.
RESULTS
This study identified 47 full-text publications, 50 conference abstracts, and 54 clinical trial registry entries describing DNA-based ocular gene therapy treatments for 16 different genetic variants. Study summaries and visual representations of safety and efficacy outcomes are presented for 20 unique full-text publications in RPE65-mediated retinal dystrophies, choroideremia, Leber hereditary optic neuropathy, rod-cone dystrophy, achromatopsia, and X-linked retinoschisis. The most common adverse events were related to lid/ocular surface/cornea abnormalities in subretinal gene therapy trials and anterior uveitis in intravitreal gene therapy trials.
CONCLUSION
There is a high degree of variability in ocular monogenic gene therapy trials with respect to study design, statistical methodology, and reporting of safety and efficacy outcomes. This review improves the accessibility and transparency in interpreting gene therapy trials to date.
Topics: Color Vision Defects; Genetic Therapy; Humans; Optic Nerve Diseases; Retina; Retinal Dystrophies
PubMed: 34906485
DOI: 10.1016/j.gim.2021.10.013 -
Sensors (Basel, Switzerland) Aug 2023Capsule endoscopy (CE) is a widely used medical imaging tool for the diagnosis of gastrointestinal tract abnormalities like bleeding. However, CE captures a huge number... (Review)
Review
Capsule endoscopy (CE) is a widely used medical imaging tool for the diagnosis of gastrointestinal tract abnormalities like bleeding. However, CE captures a huge number of image frames, constituting a time-consuming and tedious task for medical experts to manually inspect. To address this issue, researchers have focused on computer-aided bleeding detection systems to automatically identify bleeding in real time. This paper presents a systematic review of the available state-of-the-art computer-aided bleeding detection algorithms for capsule endoscopy. The review was carried out by searching five different repositories (Scopus, PubMed, IEEE Xplore, ACM Digital Library, and ScienceDirect) for all original publications on computer-aided bleeding detection published between 2001 and 2023. The Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) methodology was used to perform the review, and 147 full texts of scientific papers were reviewed. The contributions of this paper are: (I) a taxonomy for computer-aided bleeding detection algorithms for capsule endoscopy is identified; (II) the available state-of-the-art computer-aided bleeding detection algorithms, including various color spaces (RGB, HSV, etc.), feature extraction techniques, and classifiers, are discussed; and (III) the most effective algorithms for practical use are identified. Finally, the paper is concluded by providing future direction for computer-aided bleeding detection research.
Topics: Humans; Capsule Endoscopy; Computers; Computer Systems; Algorithms; Hemorrhage
PubMed: 37631707
DOI: 10.3390/s23167170