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Scandinavian Journal of Gastroenterology 2023Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic vascular anomaly mainly affecting the skin, central nervous system and gastrointestinal tracts. Its clinical...
BACKGROUND
Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic vascular anomaly mainly affecting the skin, central nervous system and gastrointestinal tracts. Its clinical presentation and characteristics in adult patients are unclear.
AIM
To clarify the characteristics of BRBNS in adult patients with a focus on gastrointestinal symptoms.
METHODS
Following the PRISMA Extension for Scoping Reviews, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including 'Blue rubber bleb nevus syndrome' from their inception to 28 December 2021.
RESULTS
Ninety-nine articles, including 3 observational studies and 101 cases from case reports and series, were included. Observational studies were consistently with small sample sizes, and there was only one prospective study to show the effectiveness of sirolimus in BRBNS. Common clinical presentations included anemia (50.5%) and melena (26.5%). While skin findings were known to be representative of BRBNS, only 57.4% had known vascular malformation. The diagnosis was primarily made clinically, with only 1% being diagnosed with BRBNS through genetic sequencing. Distribution of BRBNS-related lesions were variable, but predominantly oral (55.9%), followed by small bowel (49.5%), colorectal (35.6%) and stomach (26.7%) vascular malformations.
CONCLUSION
While it has been underrecognized, adult BRBNS could be a culprit for refractory microcytic anemia or occult gastrointestinal bleeding. Further studies are critical to establish a uniform understanding of diagnosis and treatment for those with adult BRBNS. The utility of genetic testing in adult BRBNS diagnosis and the patient characteristics that may benefit from sirolimus, a potentially curative agent, remain to be clarified.
Topics: Humans; Adult; Prospective Studies; Skin Neoplasms; Gastrointestinal Neoplasms; Sirolimus; Anemia
PubMed: 37211745
DOI: 10.1080/00365521.2023.2214263 -
Expert Review of Gastroenterology &... May 2019To review all the reported literature on acute esophageal necrosis.
BACKGROUND
To review all the reported literature on acute esophageal necrosis.
RESEARCH METHODS
Databases were searched using the special Medical Subject Heading (MeSH) terms. All the available reported cases of acute esophageal necrosis were analyzed.
RESULTS
A total of 154 cases were identified and 130 cases were analyzed. The mean age of presentation was 61 years, and 70% of cases were males. The most common presenting symptoms were hematemesis in 66%, shock in 36%, melena in 33%, abdominal or substernal pain in 28%. The most common comorbidities reported were diabetes in 38%, hypertension in 37%, alcohol abuse in 25%, and chronic kidney disease in 16%. On upper endoscopy, 51% had a distal disease, 36% had pan esophageal, and only 2% had a proximal disease. 84% of patients were treated with IV Proton Pump Inhibitors, 22% received transfusions, 23% got antibiotics for underlying sepsis, 14% also received sucralfate, and 4% required surgery for treatment. The mortality rate was 32%, while perforation was reported in 5% and stricture formation reported in 9% of patients.
CONCLUSIONS
Patients with acute esophageal necrosis can have a favorable outcome if treated appropriately.
Topics: Acute Disease; Comorbidity; Esophageal Diseases; Esophagus; Female; Humans; Male; Middle Aged; Necrosis; Risk Factors; Treatment Outcome
PubMed: 30933549
DOI: 10.1080/17474124.2019.1601555 -
Hormone Research in Paediatrics 2018There is a paucity of data on gastrointestinal (GI) vascular abnormalities in patients with Turner's syndrome (TS). Therefore, this literature review aimed to...
BACKGROUND/AIM
There is a paucity of data on gastrointestinal (GI) vascular abnormalities in patients with Turner's syndrome (TS). Therefore, this literature review aimed to characterize the nature of GI vascular malformations in patients with TS, their localization in the GI tract, and their clinical and laboratory manifestations.
METHODS
A systematic search for articles was conducted using Medline and Embase (until August 2017). A manual search of the references of the included papers was performed to identify any potential additional references. We also conducted a retrospective chart review of all patients with TS and GI bleeding who were hospitalized at our institution.
RESULTS
A total of 29 articles published between 1947 and 2015 that described 39 cases were reviewed. Additionally, we included 2 patients who were hospitalized at our institution. The median age of patients with TS was 15 years (range: 0.1-57 years). Iron deficiency anaemia (35/40), haematochezia (15/37), and melaena (14/36) were the most common symptoms. Abnormal GI vessels occurred throughout the entire bowel, with a predilection for the small intestine (72%, 29/40). The most common abnormal vessels (65%, 24/37) were telangiectasias and dilated veins.
CONCLUSION
Telangiectasias and dilated veins of the small intestine were the most commonly seen abnormal GI vessels in patients with TS.
Topics: Adolescent; Adult; Case-Control Studies; Child; Child, Preschool; Dilatation, Pathologic; Female; Gastrointestinal Tract; Humans; Infant; Male; Middle Aged; Telangiectasis; Turner Syndrome
PubMed: 30041185
DOI: 10.1159/000490425 -
Effective Clinical Practice : ECP 2001Dietary restriction is often recommended during fecal occult blood testing (FOBT) as a means of increasing test accuracy, but concern surrounds whether such restriction... (Meta-Analysis)
Meta-Analysis
CONTEXT
Dietary restriction is often recommended during fecal occult blood testing (FOBT) as a means of increasing test accuracy, but concern surrounds whether such restriction also reduces the chance that patients will complete the test.
PURPOSE
We conducted a systematic review and meta-analysis to determine if advice about dietary restrictions affects the rate of completion of FOBT and the rate of positive results.
METHODS
We searched the MEDLINE database and hand-searched the bibliographies of other systematic reviews and clinical practice guidelines to identify randomized trials of advice to perform dietary restriction during FOBT. We included only trials that reported the proportion of patients who completed the occult blood tests (completion rate). When such information was available, we also recorded the proportion of patients who had positive test results (positivity rate).
RESULTS
Five randomized trials met our inclusion criteria. All used guaiac-based Hemoccult tests; none reported results from rehydrated test slides. In four trials, there was little or no difference in test completion between patients assigned to dietary restriction and those with no restriction. In one small trial that used an especially restrictive diet, completion was 21 percentage points lower in the restricted group. Positivity rates were reported in four trials, none of which found a statistically significant difference between groups. Meta-analysis showed no difference in the summary positivity rate between those assigned to dietary restriction versus those not restricted (difference in positivity rate, 0%; 95% CI, -1% to 1%).
CONCLUSIONS
Available data suggest that advice to perform modest dietary restriction during unrehydrated FOBT does not affect the completion rate, but more severe restrictions may. Dietary restriction also does not appear to affect positivity rates. On the basis of these data, physicians do not need to advise patients to restrict their diet for nonrehydrated FOBTs.
Topics: Aged; Colonic Neoplasms; Diet; False Negative Reactions; False Positive Reactions; Female; Humans; Male; Mass Screening; Melena; Middle Aged; Occult Blood; Patient Compliance; Randomized Controlled Trials as Topic
PubMed: 11525101
DOI: No ID Found