scholarly journals Fatal Tension Hemothorax Combined With Exanguination: A Rare Complication of Neurofibromatosis

2019 ◽  
Vol 3 (4) ◽  
pp. 364-368
Author(s):  
Roz Bidad ◽  
Caroline Hall ◽  
Eike Blohm
Keyword(s):  
Rare Complication ◽  
Vascular Complications ◽  
Artery Aneurysm ◽  
Aneurysm Rupture ◽  
Autosomal Dominant Disorder ◽  
Typically Develop ◽  
Café Au Lait Spots ◽  
Arterial Ectasia

Neurofibromatosis (NF) is a common autosomal dominant disorder that can be subdivided into type 1, type 2, and schwannomatosis. Patients with NF1 typically develop café-au-lait spots, scoliosis, and benign neurofibromas. In addition, NF1 predisposes to vascular complications including stenosis, arterial ectasia, and aneurysms. Here, we report the case of an otherwise healthy 32-year-old man who developed a fatal tension hemothorax due to vertebral artery aneurysm rupture. Based on the available literature, we discuss the presentation, workup, and available therapeutic approaches to this complication of neurofibromatosis.

Relationship of Soluble RAGE and RAGE Ligands HMGB1 and EN-RAGE to Endothelial Dysfunction in Type 1 and Type 2 Diabetes Mellitus

2012 ◽  
Vol 120 (05) ◽  
pp. 277-281 ◽  
Author(s):  
J. Škrha Jr ◽  
M. Kalousová ◽  
J. Švarcová ◽  
A. Muravská ◽  
J. Kvasnička ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Endothelial Dysfunction ◽  
Advanced Glycation Endproducts ◽  
Vascular Complications ◽  
Diabetic Patients ◽  
Glycation Endproducts ◽  
Diabetic Vascular Complications ◽  
Soluble Rage

AbstractReceptor for advanced glycation endproducts (RAGE) plays the essential role in the pathogenesis of diabetic vascular complications. The aim of the study was to compare concentration of soluble RAGE and its ligands (EN-RAGE and HMGB1) with different biochemical parameters in Type 1 (T1DM) and Type 2 (T2DM) diabetes mellitus.Total number of 154 persons (45 T1DM, 68 T2DM, 41 controls) was examined and concentrations of sRAGE, EN-RAGE and HMGB1 were measured and compared to diabetes control, albuminuria, cell adhesion molecules and metalloproteinases (MMPs).Mean serum sRAGE concentration was higher in T1DM as compared to controls (1137±532 ng/l vs. 824±309 ng/l, p<0.01). Similarly, EN-RAGE was significantly higher in both diabetic groups (p<0.001) and HMGB1 concentrations were elevated in T2DM patients (p<0.01). Significant relationship was found between MMP9 and HMGB1 and EN-RAGE in diabetic patients. Inverse relationship was observed between MMP2 and MMP9 in both types of diabetic patients (r= − 0.602, p<0.002 and r= − 0.771, p<0.001). Significant positive correlation was found between sRAGE and ICAM-1, VCAM-1 or vWF (p<0.01 to p<0.001).We conclude that serum sRAGE and RAGE ligands concentrations reflect endothelial dysfunction developing in diabetes.

scholarly journals The total prevalence of diagnosed diabetes and the quality of diabetes care for the adult population in Salten, Norway

2020 ◽  
pp. 140349482095100
Author(s):  
Kristina B. Slåtsve ◽  
Tor Claudi ◽  
Knut Tore Lappegård ◽  
Anne K. Jenum ◽  
Marthe Larsen ◽  
...  
Keyword(s):  
Risk Factors ◽  
Type 2 Diabetes ◽  
Vascular Complications ◽  
Haemoglobin A1c ◽  
Total Prevalence ◽  
Quality Of Diabetes Care ◽  
Diagnosed Diabetes

Objective: To assess the total prevalence of types 1 and 2 diabetes and to describe and compare cardiovascular risk factors, vascular complications and the quality of diabetes care in adults with types 1 and 2 diabetes in Salten, Norway. Research design and methods: Cross-sectional study including all patients with diagnosed diabetes in primary and specialist care in Salten, 2014 (population 80,338). Differences in cardiovascular risk factors, prevalence of vascular complications and attained treatment targets between diabetes types were assessed using regression analyses. Results: We identified 3091 cases of diabetes, giving a total prevalence in all age groups of 3.8%, 3.4% and 0.45% for types 2 and 1 diabetes, respectively. In the age group 30–89 years the prevalence of type 2 diabetes was 5.3%. Among 3027 adults aged 18 years and older with diabetes, 2713 (89.6%) had type 2 and 304 (10.0%) type 1 diabetes. The treatment target for haemoglobin A1c (⩽7.0%/53 mmol/mol) was reached in 61.1% and 22.5% of types 2 and 1 diabetes patients, respectively. After adjusting for age, sex and diabetes duration we found differences between patients with types 2 and 1 diabetes in mean haemoglobin A1c (7.1% vs. 7.5%, P<0.001), blood pressure (136/78 mmHg vs. 131/74 mmHg, P<0.001) and prevalence of coronary heart disease (23.1% vs. 15.8%, P<0.001). Conclusions: The prevalence of diagnosed type 2 diabetes was slightly lower than anticipated. Glycaemic control was not satisfactory in the majority of patients with type 1 diabetes. Coronary heart disease was more prevalent in patients with type 2 diabetes.

scholarly journals Bilateral Spontaneous Perirenal Hemorrhage in an Acquired Cystic Kidney Disease Hemodialysis Patient

2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Daigoro Hirohama ◽  
Hiroshi Miyakawa
Keyword(s):  
Kidney Disease ◽  
Renal Artery ◽  
Rare Complication ◽  
Artery Aneurysm ◽  
Arterial Embolization ◽  
Aneurysm Rupture ◽  
Cystic Kidney Disease ◽  
Cystic Kidney ◽  
Acquired Cystic Kidney Disease ◽  
Perirenal Hemorrhage

Acquired cystic kidney disease (ACKD) is a well-known late stage complication of chronic kidney disease. Cysts tend to grow with time on dialysis and could lead to malignant transformation, and intra- or perirenal hemorrhage is a rare complication of ACKD. Here we describe one case of bilateral spontaneous perirenal hemorrhage of ACKD in a 44-year-old man, on hemodialysis for 15 years. One was due to cyst rupture, and the other was due to aneurism rupture, both were controlled with transcatheter arterial embolization. In renal arteriography at the second rupture, we demonstrated extravasation from an aneurysm being present at the periphery of right renal artery. Several spontaneous perirenal hemorrhage cases were reported but its clinical information is limited, moreover, bilateral cases were extremely rare. Furthermore, to our knowledge, this is the first report of spontaneous perirenal hemorrhage caused by intraparenchymal renal artery aneurysm rupture in ACKD patients. We report this case because of its rarity and significance with respect to the complication of dialysis patients, review reported bilateral cases, and discuss some clinical characteristics.

A clinical, genetic and audiological study of patients and families with bilateral acoustic neurofibromatosis

1993 ◽  
Vol 107 (1) ◽  
pp. 6-11 ◽  
Author(s):  
W. J. Neary ◽  
V. E. Newton ◽  
M. Vidler ◽  
R. T. Ramsden ◽  
R. H. Lye ◽  
...  
Keyword(s):  
Autosomal Dominant ◽  
Neurofibromatosis Type ◽  
Team Approach ◽  
Clinical Genetic ◽  
Acoustic Neuromas ◽  
Café Au Lait Spots ◽  
Lisch Nodules ◽  
Hereditary Disorders

AbstractThe neurofibromatoses consist of at least two distinct autosomal dominant hereditary disorders. Neurofibromatosis type 1 (NF1) is due to a lesion on chromosome 17q. Neurofibromatosis type 2 (NF2) is caused by a defect on chromosome 22q. The hallmark of NF2 is the development, in the second and third decades, of bilateral acoustic neuromas. NF1 is characterized by the appearance of cafe-au-lait spots and neurofibromas in addition to iris hamartomas, or Lisch nodules, of the eye, during the first and second decades.Ten families were personally studied. A total of 16 members were found to be affected with NF2. A protocol for evaluation and review of subjects and relatives of NF2 families is proposed. A team approach, coordinating the expertise of multiple specialties is recommended.

scholarly journals The protean role of haptoglobin and haptoglobin genotypes on vascular complications in diabetes mellitus

2018 ◽  
Vol 25 (14) ◽  
pp. 1502-1519 ◽  
Author(s):  
Rinkoo Dalan ◽  
Goh Liuh Ling
Keyword(s):  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Vitamin E ◽  
Clinical Studies ◽  
Vascular Complications ◽  
Cardiovascular Complications ◽  
Diabetes Patients

Introduction and background Haptoglobin (Hp) is considered to be an antioxidant and protective against cardiovascular complications. Polymorphisms in the Hp gene interact with diabetes mellitus to affect the risk of vascular complications. Methods We review the updated literature about the protean role of Hp and Hp genotypes spanning genomics, molecular, translational and clinical studies. We searched Pubmed, SCOPUS and Google Scholar for all articles using the keywords: haptoglobin and/or haptoglobin polymorphism and diabetes. We review the diverse Hp genotypes, phenotypes and the impact on diabetes complications, including lessons from animal models and in vitro models. We describe the clinical studies on the associations of Hp genotypes with vascular complications in type 1 and type 2 diabetes comprehensively. We review the studies looking at vitamin E supplementation in a personalized manner in Hp2-2 diabetes individuals. Results and conclusion Hp genotypes have evolved as a result of deletions in the traditional Hp genes. The Hp genotypes have been associated with microvascular and macrovascular complications in type 1 diabetes mellitus but the association in type 2 diabetes is more consistent with cardiovascular complications. A preferential benefit of vitamin E and other antioxidants in the Hp2-2 genotype for cardiovascular complications in type 2 diabetes has been seen presumably secondary to interaction with high-density lipoprotein function. Hence, the Hp genotype can be used to personalize antioxidant therapeutics in diabetes patients. These results need to be corroborated in large, global, pragmatic, prospective, cardiovascular outcome trials in type 2 diabetes patients.

scholarly journals Splenic Artery Aneurysm Case Report

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Pouya Abhari ◽  
Sina Abhari ◽  
Anwar Jackson ◽  
Ahmed S. Z. Moustafa ◽  
Leo Mercer ◽  
...  
Keyword(s):  
Case Report ◽  
Mortality Rate ◽  
Splenic Artery ◽  
Rare Complication ◽  
Artery Aneurysm ◽  
Aneurysm Rupture ◽  
Splenic Artery Aneurysm ◽  
Fetal Mortality ◽  
Fetal Survival ◽  
Very High

Splenic artery aneurysm rupture is a rare complication of pregnancy with very high maternal and fetal mortality rate. In this paper, a case of splenic artery aneurysm rupture at 34 weeks of gestation with both maternal and fetal survival is presented.

scholarly journals Giant Extracranial Aneurysm of the Internal Carotid Artery in Neurofibromatosis Type 1

2012 ◽  
Vol 18 (3) ◽  
pp. 341-347 ◽  
Author(s):  
C. Moratti ◽  
T. Andersson
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Neurofibromatosis Type 1 ◽  
Internal Carotid ◽  
Artery Aneurysm ◽  
Neurofibromatosis Type ◽  
Parent Artery ◽  
Autosomal Dominant Disorder ◽  
Internal Carotid Artery Aneurysm

Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by cutaneous pigmentations, neurofibromas, Lisch nodules and neuroectodermal tumors. Supra-aortic vessel aneurysms may affect patients with NF-1 and can be associated with rupture, ischemic complications and compression symptoms. We describe a 48-year-old woman with NF-1 and an extracranial 3×5 cm right internal carotid artery aneurysm. After balloon test occlusion the patient was treated with parent artery sacrifice which led to significant shrinkage on follow-up MR and reduction of compression symptoms. The literature concerning internal carotid artery aneurysms associated with NF-1 is reviewed evaluating the possible therapeutic options.

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