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Medicina (Kaunas, Lithuania) May 2024: Functional gastric stenosis, a consequence of sleeve gastrectomy, is defined as a rotation of the gastric tube along its longitudinal axis. It is brought on by gastric...
: Functional gastric stenosis, a consequence of sleeve gastrectomy, is defined as a rotation of the gastric tube along its longitudinal axis. It is brought on by gastric twisting without the anatomical constriction of the gastric lumen. During endoscopic examination, the staple line is deviated with a clockwise rotation, and the stenosis requires additional endoscopic manipulations for its transposition. Upper gastrointestinal series show the gastric twist with an upstream dilatation of the gastric tube in some patients. Data on its management have remained scarce. The objective was to assess the efficacy and safety of endoscopic balloon dilatation in the management of functional post-sleeve gastrectomy stenosis. : Twenty-two patients with functional post-primary-sleeve-gastrectomy stenosis who had an endoscopic balloon dilatation between 2017 and 2023 were included in this retrospective study. Patients with alternative treatment plans and those undergoing endoscopic dilatation for other forms of gastric stenosis were excluded. The clinical outcomes were used to evaluate the efficacy and safety of balloon dilatation in the management of functional gastric stenosis. : A total of 45 dilatations were performed with a 30 mm balloon in 22 patients (100%), a 35 mm balloon in 18 patients (81.82%), and a 40 mm balloon in 5 patients (22.73%). The patients' clinical responses after the first balloon dilatation were a complete clinical response (4 patients, 18.18%), a partial clinical response (12 patients, 54.55%), and a non-response (6 patients, 27.27%). Nineteen patients (86.36%) had achieved clinical success at six months. Three patients (13.64%) who remained symptomatic even after achieving the maximal balloon dilation of 40 mm were considered failure of endoscopic dilatation, and they were referred for surgical intervention. No significant adverse events were found during or following the balloon dilatation. : Endoscopic balloon dilatation is an effective and safe minimally invasive procedure in the management of functional post-sleeve-gastrectomy stenosis.
Topics: Humans; Male; Female; Gastrectomy; Middle Aged; Retrospective Studies; Dilatation; Adult; Treatment Outcome; Constriction, Pathologic; Postoperative Complications
PubMed: 38793016
DOI: 10.3390/medicina60050833 -
Novel Gene Mutation c.118G>A Causing Keratinocyte and Cardiomyocyte Disconnection in Darier Disease.Biomedicines May 2024Darier disease (DD) is an autosomal dominant disorder due to pathogenic variants of the gene that causes an isolated skin manifestation based on keratinocyte...
Darier disease (DD) is an autosomal dominant disorder due to pathogenic variants of the gene that causes an isolated skin manifestation based on keratinocyte disconnection and apoptosis. Systemic manifestations of DD have not been demonstrated so far, although a high incidence of neuropsychiatric syndromes suggests an involvement of the central nervous system. We report that the pathogenic ATP2A2 gene variant c.118G>A may cause cardiac involvement in patients with DD, consisting of keratinocyte and cardiomyocyte disconnection. Their common pathologic pathway, still unreported, was documented by both skin and left ventricular endomyocardial biopsies because cardiac dilatation and dysfunction appeared several decades after skin manifestations. Keratinocyte disconnection was paralleled by cardiomyocyte separation at the lateral junction. Cardiomyocyte separation was associated with cell disarray, sarcoplasmic reticulum dilatation, and increased myocyte apoptosis. Clinically, hyperkeratotic skin papules are associated with chest pain, severe muscle exhaustion, and ventricular arrhythmias that improved following administration of aminophylline, a phosphodiesterase inhibitor enhancing SERCA2 protein phosphorylation. Cardiac pathologic changes are similar to those documented in the skin, including cardiomyocyte disconnection that promotes precordial pain and cardiac arrhythmias. Phosphodiesterase inhibitors that enhance SERCA2 protein phosphorylation may substantially attenuate the symptoms.
PubMed: 38791022
DOI: 10.3390/biomedicines12051060 -
Arteriosclerosis, Thrombosis, and... Jul 2024β-aminopropionitrile (BAPN) is a pharmacological inhibitor of LOX (lysyl oxidase) and LOXLs (LOX-like proteins). Administration of BAPN promotes aortopathies, although...
BACKGROUND
β-aminopropionitrile (BAPN) is a pharmacological inhibitor of LOX (lysyl oxidase) and LOXLs (LOX-like proteins). Administration of BAPN promotes aortopathies, although there is a paucity of data on experimental conditions to generate pathology. The objective of this study was to define experimental parameters and determine whether equivalent or variable aortopathies were generated throughout the aortic tree during BAPN administration in mice.
METHODS
BAPN was administered in drinking water for a period ranging from 1 to 12 weeks. The impacts of BAPN were first assessed with regard to BAPN dose, and mouse strain, age, and sex. BAPN-induced aortic pathological characterization was conducted using histology and immunostaining. To investigate the mechanistic basis of regional heterogeneity, the ascending and descending thoracic aortas were harvested after 1 week of BAPN administration before the appearance of overt pathology.
RESULTS
BAPN-induced aortic rupture predominantly occurred or originated in the descending thoracic aorta in young C57BL/6J or N mice. No apparent differences were found between male and female mice. For mice surviving 12 weeks of BAPN administration, profound dilatation was consistently observed in the ascending region, while there were more heterogeneous changes in the descending thoracic region. Pathological features were distinct between the ascending and descending thoracic regions. Aortic pathology in the ascending region was characterized by luminal dilatation and elastic fiber disruption throughout the media. The descending thoracic region frequently had dissections with false lumen formation, collagen deposition, and remodeling of the wall surrounding the false lumen. Cells surrounding the false lumen were predominantly positive for α-SMA (α-smooth muscle actin). One week of BAPN administration compromised contractile properties in both regions equivalently, and RNA sequencing did not show obvious differences between the 2 aortic regions in smooth muscle cell markers, cell proliferation markers, and extracellular components.
CONCLUSIONS
BAPN-induced pathologies show distinct, heterogeneous features within and between ascending and descending aortic regions in mice.
Topics: Animals; Aminopropionitrile; Aorta, Thoracic; Female; Male; Mice, Inbred C57BL; Disease Models, Animal; Aortic Rupture; Mice; Vascular Remodeling; Dilatation, Pathologic; Muscle, Smooth, Vascular; Age Factors; Time Factors; Sex Factors; Cell Proliferation; Protein-Lysine 6-Oxidase
PubMed: 38779856
DOI: 10.1161/ATVBAHA.123.320402 -
Experimental and Clinical... Apr 2024Biliary strictures afterlivertransplant are amenable to endoscopic dilatation or percutaneous dilatation and stenting in most cases. In rare cases, for recurrence or...
Biliary strictures afterlivertransplant are amenable to endoscopic dilatation or percutaneous dilatation and stenting in most cases. In rare cases, for recurrence or tight stricture, surgery is required, and hepaticojejunostomy is the favored procedure. We report a case of posttransplant stricture in a duct-to-duct anastomosis that could not be accessed due to prior gastric bypass. Despite multiple percutaneous transhepatic cholangiography dilatations, the stricture recurred, and the patient was taken up for bilioenteric bypass. During surgery, dense adhesions in the infracolic compartment with chronically twisted jejunal loops, due to prior mini gastric bypass, were encountered, which prevented the creation of a jejunal Roux limb. Hepaticoduodenostomy was performed with no recurrence of stricture at 12 months. Hepaticoduodenostomy is a viable option for surgical management of recurrent biliary strictures, especially in a setting of prior bariatric/diversion procedures.
Topics: Humans; Liver Transplantation; Recurrence; Constriction, Pathologic; Treatment Outcome; Duodenostomy; Reoperation; Cholestasis; Middle Aged; Anastomosis, Surgical; Female; Male; Cholangiography
PubMed: 38742323
DOI: 10.6002/ect.2024.0070 -
BMC Pediatrics May 2024Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to...
BACKGROUND
Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to adults. This study aimed to investigate the application of various assessment indicators of AD in Chinese children and adolescents with TS.
METHODS
This study included TS patients admitted to Shenzhen Children's Hospital from 2017 to 2022. Cardiovascular lesions were diagnosed by experienced radiologists. Patients without structural heart disease were divided into different body surface area groups, then the Chinese TS population Z-score (CHTSZ-score) of the ascending aorta was calculated and compared with other indicators such as aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and TSZ-score (Quezada's method).
RESULTS
A total of 115 TS patients were included, with an average age of 10.0 ± 3.7 years. The incidences of the three most serious cardiovascular complications were 9.6% (AD), 10.4% (coarctation of the aorta, CoA), and 7.0% (bicuspid aortic valve, BAV), respectively. The proportion of developing AD in TS patients aged ≥ 10 years was higher than that in those < 10 years old (16.6% vs. 1.8%, P = 0.009), and the proportion of patients with CoA or BAV who additionally exhibited AD was higher than those without these conditions (31.6% vs. 5.2%, P < 0.001). The ASI, A/D ratio, TSZ-score, and CHTSZ-score of the 11 patients with AD were 2.27 ± 0.40 cm/m, 1.90 ± 0.37, 1.28 ± 1.08, and 3.07 ± 2.20, respectively. Among the AD patients, only 3 cases had a TSZ-score ≥ 2, and 2 cases had a TSZ-score ≥ 1. However, based on the assessment using the CHTSZ-score, 6 patients scored ≥ 2, and 5 patients scored ≥ 1. In contrast, the TSZ-score generally underestimated the aortic Z-scores in Chinese children with TS compared to the CHTSZ-score.
CONCLUSIONS
The applicability of ASI and A/D ratio to children with TS is questionable, and racial differences can affect the assessment of TSZ-score in the Chinese population. Therefore, establishing the CHTSZ-score specifically tailored for Chinese children and adolescents is of paramount importance.
Topics: Humans; Turner Syndrome; Child; Adolescent; Female; China; Dilatation, Pathologic; Male; Retrospective Studies; Aorta; Aortic Coarctation; Bicuspid Aortic Valve Disease; Child, Preschool; Incidence; East Asian People
PubMed: 38720245
DOI: 10.1186/s12887-024-04783-2 -
Current Opinion in Ophthalmology Jul 2024To review corneal crosslinking for keratoconus and corneal ectasia, and recent developments in the field. This study will review the mechanism of crosslinking, clinical... (Review)
Review
PURPOSE OF REVIEW
To review corneal crosslinking for keratoconus and corneal ectasia, and recent developments in the field. This study will review the mechanism of crosslinking, clinical approaches, current results, and potential future innovations.
RECENT FINDINGS
Corneal crosslinking for keratoconus was first approved by U.S. FDA in 2016. Recent studies have confirmed the general long-term efficacy of the procedure in decreasing progression of keratoconus and corneal ectasia. New types of crosslinking protocols, such as transepithelial treatments, are under investigation. In addition, adjunctive procedures have been developed to improve corneal contour and visual function in these patients.
SUMMARY
Crosslinking has been found to be well tolerated and effective with the goal of decreasing progression of ectatic corneal diseases, keratoconus and corneal ectasia after refractive surgery. Studies have shown its long-term efficacy. New techniques of crosslinking and adjunctive procedures may further improve treatments and results.
Topics: Keratoconus; Humans; Cross-Linking Reagents; Riboflavin; Photosensitizing Agents; Photochemotherapy; Dilatation, Pathologic; Collagen; Ultraviolet Rays; Corneal Stroma
PubMed: 38700496
DOI: 10.1097/ICU.0000000000001056 -
World Journal of Surgery Feb 2024The diseased bile duct in bilobar congenital biliary dilatation is extensive and often requires major hepatectomy or liver transplantation associated with a higher risk.... (Comparative Study)
Comparative Study
BACKGROUND
The diseased bile duct in bilobar congenital biliary dilatation is extensive and often requires major hepatectomy or liver transplantation associated with a higher risk. We aimed to evaluate the safety and benefit of modified mesohepatectomy, in comparison with trisectionectomy, to treat bilobar congenital biliary dilatation.
METHODS
This study included 28 patients with type IV and V bilobar congenital biliary dilatation. An innovative mesohepatectomy comprising the hepatectomy technique beyond the P/U point and bile duct shaping was applied to 14 patients to address the extensively diseased bile duct and difficulty in hepaticojejunostomy. Another 14 patients received trisectionectomy. The perioperative and long-term outcomes of these patients were compared.
RESULTS
The ratio of residual liver volume to standard liver volume in the mesohepatectomy group was higher (78.68% vs. 40.90%, p = 0.005), while the resection rate of the liver parenchyma was lower (28.25% vs. 63.97%, p = 0.000), than that in trisectionectomy group. The mesohepatectomy group had a lower severe complication (>Clavein III, 0% vs. 57.70%, p = 0.019) and incidence of posthepatectomy liver failure (7.14% vs. 42.86%, p = 0.038). No significant difference was observed in blood loss and bile leakage (p > 0.05). All the patients in the mesohepatectomy group achieved optimal results in the long-term follow-up.
CONCLUSIONS
mesohepatectomy provides an efficient treatment option for bilobar congenital biliary dilatation and can achieve radical resection, retain more liver parenchyma, and reduce the difficulty of hepaticojejunostomy, especially for patients that are not eligible for major hepatectomy and liver transplantation.
Topics: Humans; Hepatectomy; Male; Female; Treatment Outcome; Retrospective Studies; Dilatation, Pathologic; Infant; Postoperative Complications; Child, Preschool
PubMed: 38686786
DOI: 10.1002/wjs.12036 -
International Journal of Cardiology Jul 2024
Topics: Humans; Transcatheter Aortic Valve Replacement; Aortic Valve Stenosis; Aorta; Dilatation, Pathologic; Male; Aged; Aged, 80 and over; Female
PubMed: 38663807
DOI: 10.1016/j.ijcard.2024.132089 -
Journal of Pediatric Gastroenterology... Jun 2024Previous studies have shown rates of surgical resection of up to 41% in stricturing pediatric Crohn's disease (CD). In this retrospective multicenter study, our aims...
OBJECTIVES
Previous studies have shown rates of surgical resection of up to 41% in stricturing pediatric Crohn's disease (CD). In this retrospective multicenter study, our aims were to identify clinical risk factors and magnetic resonance enterography (MRE) features of small bowel strictures associated with surgery.
METHODS
Pediatric patients with symptomatic stricturing small bowel CD (defined as obstructive symptoms or proximal dilatation on MRE) confirmed by MRE between 2010 and 2020 were recruited from 12 French tertiary hospitals. Patient characteristics were compared by surgical outcome multivariable Cox regression.
RESULTS
Fifty-six patients (61% boys) aged 12.2 ± 2.7 years at diagnosis of CD were included. Median duration of CD before diagnosis of stricture was 11.7 months (interquartile range [IQR]: 25-75: 1.2-29.9). Nineteen (34%) patients had stricturing phenotype (B2) at baseline. Treatments received before stricture diagnosis included MODULEN-IBD (n = 31), corticosteroids (n = 35), antibiotics (n = 10), anti-TNF (n = 27), immunosuppressants (n = 28). Thirty-six patients (64%) required surgery, within 4.8 months (IQR: 25-75: 1.8-17.3) after stricture diagnosis. Parameters associated with surgical resection were antibiotic exposure before stricture diagnosis (adjusted odds ratio [aOR]: 15.62 [3.35-72.73], p = 0.0005), Crohn's disease obstructive symptoms score (CDOS) > 4 (aOR: 3.04 [1.15-8.03], p = 0.02) and dilation proximal to stricture >28 mm (aOR: 3.62 [1.17-11.20], p = 0.03).
CONCLUSION
In this study, antibiotic treatment before stricture diagnosis, intensity of obstructive symptoms, and diameter of dilation proximal to small bowel stricture on MRE were associated with risk for surgical resection.
Topics: Humans; Crohn Disease; Male; Retrospective Studies; Female; Risk Factors; Child; Intestine, Small; Adolescent; Constriction, Pathologic; France; Magnetic Resonance Imaging; Intestinal Obstruction
PubMed: 38651614
DOI: 10.1002/jpn3.12224 -
Surgical Endoscopy Jun 2024Studies have evaluated the efficacy of endoscopic incisional therapy (EIT) for benign anastomotic strictures. We performed a systematic review and meta-analysis to... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Studies have evaluated the efficacy of endoscopic incisional therapy (EIT) for benign anastomotic strictures. We performed a systematic review and meta-analysis to evaluate stricture recurrence after EIT following esophagectomy or gastrectomy.
METHODS
A systematic search of databases was performed up to April 2nd, 2023, after selection of key search terms with the research team. Inclusion criteria included human participants undergoing EIT for a benign anastomotic stricture after esophagectomy or gastrectomy, age ≥ 18, and n ≥ 5. Our primary outcome was the incidence of stricture recurrence among patients treated with EIT compared to dilation. Our secondary outcome was the stricture-free duration after EIT and rate of adverse events. Meta-analysis was performed with RevMan 5.4.1 using a Mantel-Haenszel random-effects model. Publication bias was evaluated with funnel plots and the Egger test.
RESULTS
A total of 2550 unique preliminary studies underwent screening of abstracts and titles. This led to 33 studies which underwent full-text review and five studies met the inclusion criteria. Meta-analysis revealed reduced odds of overall stricture recurrence (OR 0.35, 95% CI 0.13-0.92, p = 0.03; I = 71%) and reduced odds of stricture recurrence among naïve strictures (OR 0.32, 95% CI 0.17-0.59, p = 0.0003; I = 0%) for patients undergoing EIT compared to dilation. There was no significant difference in the odds of stricture recurrence among recurrent strictures (OR 0.63, 95% CI 0.12-3.28, p = 0.58; I = 81%). Meta-analysis revealed a significant increase in the recurrence-free duration (MD 42.76, 95% CI 12.41-73.11, p = 0.006) among patients undergoing EIT compared to dilation.
CONCLUSION
Current data suggest EIT is associated with reduced odds of stricture recurrence among naïve anastomotic strictures. Large, prospective studies are needed to characterize the safety profile of EIT, address publication bias, and to explore multimodal therapies for refractory strictures.
Topics: Humans; Esophagectomy; Gastrectomy; Anastomosis, Surgical; Esophageal Stenosis; Postoperative Complications; Constriction, Pathologic; Recurrence; Dilatation
PubMed: 38649492
DOI: 10.1007/s00464-024-10817-8