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HCA Healthcare Journal of Medicine 2021Hemangiomas are benign vascular tumors that are common during infancy. They are most commonly noted as superficial bright red lesions on the skin but can also be found...
INTRODUCTION
Hemangiomas are benign vascular tumors that are common during infancy. They are most commonly noted as superficial bright red lesions on the skin but can also be found deeper as subcutaneous lesions. Patients with multifocal cutaneous hemangiomas are at risk of visceral involvement with the liver being most commonly affected. Most hemangiomas can be monitored clinically as they are self-limiting. Despite this, hepatic hemangiomas can have serious complications including large arteriovenous shunts leading to cardiac compromise as well as severe hepatomegaly which can cause abdominal compartment syndrome, impaired ventilation and renal vein compression.
CLINICAL FINDINGS
A six-month-old female, born full term and previously healthy, presented due to worsening abdominal distention and hepatomegaly. On examination, abdominal distention, hepatomegaly and three superficial hemangiomas on her torso and scalp were appreciated.
OUTCOME
The patient had an extensive workup which showed an elevated AFP and TSH. An abdominal ultrasound revealed numerous rounded regions of hypoechogenicity throughout the hepatic parenchyma. These findings were consistent with diffuse infantile hemangiomas; however, metastasis could not be ruled out with ultrasound alone. An MRI of the abdomen was obtained which confirmed infantile hemangiomas. The patient's MRI and lab findings are the classical findings of infantile hepatic hemangiomas. She had elevated blood pressures during the hospital course and was subsequently evaluated by cardiology for concern of cardiac compromise. Treatment with propranolol was initiated and continued upon discharge. A six month follow-up ultrasound showed significant decrease in size of the hemangiomas.
CONCLUSION
Hepatic hemangiomas should be monitored closely for serious complications. Although rare, it is important to identify which patients with multifocal cutaneous hemangiomas should be worked up for hepatic hemangiomas and their complications. It is recommended that infants younger than 6 months of age with 5 or more cutaneous hemangiomas undergo early evaluation with abdominal ultrasound.
PubMed: 37424838
DOI: 10.36518/2689-0216.1250 -
Biomolecules Mar 2022Abdominal aortic aneurysm (AAA), defined as a focal dilation of the abdominal aorta beyond 50% of its normal diameter, is a common and potentially life-threatening... (Review)
Review
Abdominal aortic aneurysm (AAA), defined as a focal dilation of the abdominal aorta beyond 50% of its normal diameter, is a common and potentially life-threatening vascular disease. The molecular and cellular mechanisms underlying AAA pathogenesis remain unclear. Healthy endothelial cells (ECs) play a critical role in maintaining vascular homeostasis by regulating vascular tone and maintaining an anti-inflammatory, anti-thrombotic local environment. Increasing evidence indicates that endothelial dysfunction is an early pathologic event in AAA formation, contributing to both oxidative stress and inflammation in the degenerating arterial wall. Recent studies utilizing single-cell RNA sequencing revealed heterogeneous EC sub-populations, as determined by their transcriptional profiles, in aortic aneurysm tissue. This review summarizes recent findings, including clinical evidence of endothelial dysfunction in AAA, the impact of biomechanical stress on EC in AAA, the role of endothelial nitric oxide synthase (eNOS) uncoupling in AAA, and EC heterogeneity in AAA. These studies help to improve our understanding of AAA pathogenesis and ultimately may lead to the generation of EC-targeted therapeutics to treat or prevent this deadly disease.
Topics: Aorta, Abdominal; Aortic Aneurysm, Abdominal; Dilatation, Pathologic; Endothelial Cells; Humans; Oxidative Stress
PubMed: 35454098
DOI: 10.3390/biom12040509 -
Nutrients Feb 2021Postprandial objective abdominal distention is frequently associated with a subjective sensation of abdominal bloating, but the relation between both complaints is... (Randomized Controlled Trial)
Randomized Controlled Trial
Postprandial objective abdominal distention is frequently associated with a subjective sensation of abdominal bloating, but the relation between both complaints is unknown. While the bloating sensation has a visceral origin, abdominal distention is a behavioral somatic response, involving contraction and descent of the diaphragm with protrusion of the anterior abdominal wall. Our aim was to determine whether abdominal distention influences digestive sensations. In 16 healthy women we investigated the effect of intentional abdominal distention on experimentally induced bloating sensation (by a meal overload). Participants were first taught to produce diaphragmatic contraction and visible abdominal distention. After a meal overload, sensations of bloating (0 to 10) and digestive well-being (-5 to + 5) were measured during 30-s. maneuvers alternating diaphragmatic contraction and diaphragmatic relaxation. Compared to diaphragmatic relaxation, diaphragmatic contraction was associated with diaphragmatic descent (by 21 + 3 mm; < 0.001), objective abdominal distension (32 + 5 mm girth increase; = 0.001), more intense sensation of bloating (7.3 + 0.4 vs. 8.0 + 0.4 score; = 0.010) and lower digestive well-being (-0.9 + 0.5 vs. -1.9 + 0.5 score; = 0.028). These results indicate that somatic postural tone underlying abdominal distention worsens the perception of visceral sensations (ClinicalTrials.gov ID: NCT04691882).
Topics: Abdomen; Adult; Cross-Over Studies; Diaphragm; Digestion; Eating; Female; Healthy Volunteers; Humans; Hyperphagia; Meals; Postprandial Period; Posture; Sensation; Thorax
PubMed: 33670508
DOI: 10.3390/nu13020658 -
Indian Pediatrics Mar 2007We present here the first case of Fanconi-Bickel syndrome, a rare type of glycogen storage disease, from India. A 17-month-old female child presented with severe growth...
We present here the first case of Fanconi-Bickel syndrome, a rare type of glycogen storage disease, from India. A 17-month-old female child presented with severe growth retardation and abdominal distention. Clinical examination revealed a "doll-like" face, massive hepatomegaly, and rickets. Laboratory investigations confirmed severe hypophosphatemic rickets and proximal renal tubular dysfunction. Liver biopsy showed glycogen accumulation in the hepatocytes.
Topics: Abdomen; Dietary Supplements; Failure to Thrive; Familial Hypophosphatemic Rickets; Fanconi Syndrome; Female; Glycogen Storage Disease; Hepatomegaly; Humans; Infant
PubMed: 17413201
DOI: No ID Found -
Cardiovascular Research Nov 2018Aortic aneurysm is a life-threatening disease due to the risk of aortic rupture. The only curative treatment available relies on surgical approaches; drug-based... (Review)
Review
Aortic aneurysm is a life-threatening disease due to the risk of aortic rupture. The only curative treatment available relies on surgical approaches; drug-based therapies are lacking, highlighting an unmet need for clinical practice. Abdominal aortic aneurysm (AAA) is frequently associated with atherosclerosis and cardiovascular risk factors including male sex, age, smoking, hypertension, and dyslipidaemia. Thoracic aortic aneurysm (TAA) is more often linked to genetic disorders of the extracellular matrix and the contractile apparatus but also share similar cardiovascular risk factors. Intriguingly, a large body of evidence points to an inverse association between diabetes and both AAA and TAA. A better understanding of the mechanisms underlying the negative association between diabetes and aortic aneurysm could help the development of innovative diagnostic and therapeutic approaches to tackle the disease. Here, we summarize current knowledge on the relationship between glycaemic parameters, diabetes, and the development of aortic aneurysm. Cellular and molecular pathways that underlie the protective effect of diabetes itself and its treatment are reviewed and discussed, along with their potential implications for clinical translation.
Topics: Animals; Aortic Aneurysm, Abdominal; Aortic Aneurysm, Thoracic; Biomarkers; Blood Glucose; Diabetes Mellitus; Dilatation, Pathologic; Disease Models, Animal; Glycated Hemoglobin; Humans; Hypoglycemic Agents; Prognosis; Protective Factors; Risk Assessment; Risk Factors; Vascular Remodeling
PubMed: 30052821
DOI: 10.1093/cvr/cvy174 -
Diabetes & Vascular Disease Research Sep 2018Diabetes mellitus appears to be negatively associated with abdominal aortic aneurysm; however, the mechanisms underlying this relationship remain poorly understood. The... (Review)
Review
INTRODUCTION
Diabetes mellitus appears to be negatively associated with abdominal aortic aneurysm; however, the mechanisms underlying this relationship remain poorly understood. The aim of this article is to provide a comprehensive review of the currently understood biological pathways underlying this relationship.
METHODS
A review of the literature ('diabetes' OR 'hyperglycaemia' AND 'aneurysm') was performed and relevant studies grouped into biological pathways.
RESULTS
This review identified a number of biological pathways through which diabetes mellitus may limit the presence, growth and rupture of abdominal aortic aneurysms. These include those influencing extracellular matrix volume, extracellular matrix glycation, the formation of advanced glycation end-products, inflammation, oxidative stress and intraluminal thrombus biology. In addition, there is an increasing evidence to suggest that the medications used to treat diabetes can also limit the development and progression of abdominal aortic aneurysms.
CONCLUSION
The negative association between diabetes and abdominal aortic aneurysm is robust. Future studies should attempt to target the pathways identified in this review to develop novel therapeutic agents aimed at slowing or even halting aneurysm progression.
Topics: Animals; Aorta, Abdominal; Aortic Aneurysm, Abdominal; Biomarkers; Blood Glucose; Diabetes Mellitus; Dilatation, Pathologic; Disease Progression; Extracellular Matrix; Glycation End Products, Advanced; Humans; Hypoglycemic Agents; Inflammation Mediators; Oxidative Stress; Risk Factors; Vascular Remodeling
PubMed: 29874945
DOI: 10.1177/1479164118780799 -
The New England Journal of Medicine Sep 2009A 67-year-old woman with type 2 diabetes mellitus undergoes extensive resection of the small bowel and right colon with a jejunostomy and colostomy because of mesenteric... (Review)
Review
A 67-year-old woman with type 2 diabetes mellitus undergoes extensive resection of the small bowel and right colon with a jejunostomy and colostomy because of mesenteric ischemia. In the surgical intensive care unit, severe systemic inflammatory response syndrome with possible sepsis develops. The patient is treated with volume resuscitation, vasopressor support, mechanical ventilation, broad-spectrum antibiotics, and intravenous insulin infusion. Low-dose tube feedings are initiated postoperatively through a nasogastric tube. However, these feedings are discontinued after the development of escalating vasopressor requirements, worsening abdominal distention, and increased gastric residual volume, along with an episode of emesis. The hospital nutritional-support service is consulted for feeding recommendations. A discussion with the patient's family reveals that during the previous 6 months, she lost approximately 15% of her usual body weight and decreased her food intake because of abdominal pain associated with eating. Her preoperative body weight was 51 kg (112 lb), or 90% of her ideal body weight. The physical examination reveals mild wasting of skeletal muscle and fat. Blood tests show hypomagnesemia, hypophosphatemia, and normal hepatic and renal function. Central venous parenteral nutrition is recommended.
Topics: Aged; Catheterization, Central Venous; Contraindications; Critical Illness; Diabetes Mellitus, Type 2; Female; Humans; Intensive Care Units; Ischemia; Mesentery; Parenteral Nutrition; Postoperative Complications; Practice Guidelines as Topic; Protein-Energy Malnutrition
PubMed: 19741230
DOI: 10.1056/NEJMct0806956 -
Journal of Vascular Surgery Feb 2014The purpose of this study was to summarize the current evidence of the association between markers of hemostasis and both the presence and size of abdominal aortic... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The purpose of this study was to summarize the current evidence of the association between markers of hemostasis and both the presence and size of abdominal aortic aneurysms (AAAs).
METHODS
A systematic review and meta-analysis was performed according to Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines by use of the search terms "aneurysm AND abdominal AND aortic AND coagulation" NOT "thoracic." Outcome data including concentration of hemostatic marker, number of patients, and significance level were recorded.
RESULTS
A total of 22 nonrandomized studies were included in the analysis, with a total of 9862 patients. Fibrinogen mean difference (MD) (0.43 g/L; 95% confidence interval [CI], 0.28-0.58 g/L; P ≤ .00001), D-dimer MD (325.82 ng/mL; 95% CI, 199.74-451.89 ng/mL; P ≤ .00001), and thrombin-antithrombin III complex MD (5.58 g/L; 95% CI, 3.34-7.83 g/L; P ≤ .0001) were significantly elevated in the presence of AAAs. Tissue plasminogen activator, prothrombin fragments F1+F2, and platelet count were not shown to be significantly different between patients with and those without AAAs. Meta-regression of studies reporting plasma D-dimer concentration and aneurysm diameter suggests a strong and significant association (r(2) = 0.94; P ≤ .0001).
CONCLUSIONS
This study suggests that the presence of AAAs is associated with increased fibrin turnover, fibrinolysis, and thrombin generation, as shown by increased levels of fibrinogen, D-dimer, and thrombin-antithrombin III complex. This is clinically relevant because markers of hemostasis are independent risk factors for cardiovascular events, highlighting the necessity of addressing all modifiable cardiovascular risk factors in patients with AAAs. Furthermore, the finding that plasma D-dimer concentration appears to have a linear relationship with aneurysm diameter may be useful as a future biomarker of AAAs.
Topics: Antithrombin III; Aorta, Abdominal; Aortic Aneurysm, Abdominal; Biomarkers; Dilatation, Pathologic; Fibrin Fibrinogen Degradation Products; Fibrinogen; Hemostasis; Humans; Peptide Hydrolases; Prognosis; Risk Factors
PubMed: 24461868
DOI: 10.1016/j.jvs.2013.10.088 -
Journal of Education & Teaching in... Jan 2022A 5-month-old female presented to the emergency department with worsening abdominal distention and postprandial emesis. Point-of-care ultrasound (POCUS) demonstrated...
UNLABELLED
A 5-month-old female presented to the emergency department with worsening abdominal distention and postprandial emesis. Point-of-care ultrasound (POCUS) demonstrated extensive abdominal free fluid with a large, heterogeneous mass in the suprapubic region. This was confirmed on computed tomography (CT) of the abdomen and pelvis. The patient was urgently taken to the operating room for exploratory laparotomy where a ruptured tumor was discovered. The patient was eventually diagnosed with ovarian juvenile granulosa cell tumor. POCUS can be vital in guiding the recognition of rare pediatric conditions in the emergency department and should be considered in patients where there is concern for an acute abdomen.
TOPICS
Abdominal pain, ascites, ovarian juvenile granulosa cell tumor, point-of-care ultrasound.
PubMed: 37483405
DOI: 10.21980/J8035H -
Digestive Surgery 2012The occurrence of anastomotic stricture at the level of the rectum gives rise to three broad therapeutic options, namely major pelvic and abdominal revisional surgery,... (Review)
Review
BACKGROUND
The occurrence of anastomotic stricture at the level of the rectum gives rise to three broad therapeutic options, namely major pelvic and abdominal revisional surgery, faecal diversion (stoma), or local revision by transanal approaches (including endoscopic and fluoroscopic). This article updates the current evidence and focuses on the results of the balloon dilatation technique.
METHODS
A Medline search was carried out using the search terms (dilatation OR dilatation) AND (stricture OR strictures OR stenosis OR stenotic) AND (rectum OR rectal). In an effort to lessen publication bias, articles included at least 10 patients who were consecutively referred for treatment.
RESULTS/CONCLUSION
This review would suggest that probably relatively short strictures have been chosen for balloon dilatation and that the results have had a very low major morbidity (0.45%) and mortality (0%) rate.
Topics: Anastomosis, Surgical; Constriction, Pathologic; Dilatation; Humans; Proctoscopy; Rectum; Treatment Outcome
PubMed: 22922944
DOI: 10.1159/000341657