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What is Blue Rubber Bleb Nevus Syndrome or BRBNS?
The Blue Rubber Bleb Nevus Syndrome or BRBNS is an super rare vascular disorder that affects the parentage vessels around the regions of the unlike internal organs of the human body or areas just under the skin . One of the vulgar symptom of Blue Rubber Bleb Nevus Syndrome or BRBNS is the presence of pelt lesion either at birth meter or even during the puerility period . These are venous malformations that are multifocal in their nature . Blue Rubber Bleb Nevus Syndrome often emerges due to abnormal maturation of the blood vessel at the embryonal stage . The condition was first explain in the class 1860 by Gascoyne . However , it was not till the yr 1958 when the construct was further looked into by William Bennett Bean , which is why the condition is also pertain to as Bean Syndrome .
Symptoms of Blue Rubber Bleb Nevus Syndrome or BRBNS
Blue Rubber Bleb Nevus Syndrome or BRBNS is characteristically present with multiple skin lesions which are soft in their nature . The lesion are tender in texture and are fill with blood(1 ) . These lesion can be of different colouration like calamitous , red - over-embellished , red or even dark blue in people of colour . They can be compact quite well and can be found in the sphere of the soles and the trunks of the feet and can also be found on the upper arm as well . The skin lesion can spring up over a full point of time and can increase in their size . However , they have not been report to be cancerous in their nature . The touched line watercraft are commonly found in the arena of the GI tract with the minuscule intestine being one of the most common places where they are establish . These lesion that can be find in the diminished intestine can bleed and can lead to cases of anaemia in the affected role . The intensity of anemia can differ from patient to patient . In this condition , smoothing iron replacement is one of the most take procedures of treatment . In several cases , blood transfusion could also be required for a proper intervention process . Gastrointestinal lesions can also cause problem for the procedure of bowel movement and can also affect muscle formation in the body(2 ) .
Causes Blue Rubber Bleb Nevus Syndrome or BRBNS
The causes of Blue Rubber Bleb Nevus Syndrome or BRBNS are rather erratic . The precise reason behind the experimental condition is not yet very well know by the medical fraternity . However , the problem could be a rare genetic upset where it happens to be autosomal in nature . In other Son , if the small fry inherits an moved gene from any one of its parents then the child may have the consideration . It has been examine that patient with the condition did have people with the same problem in their kinsperson . Blue Rubber Bleb Nevus Syndrome affects both females and males in an equal fashion . So far 150 cases of Blue Rubber Bleb Nevus Syndrome have been reported and they do not belong to any individual part of the world in special . multitude from every ethnicity have been name with this syndrome(1 ) .
Epidemiology of Blue Rubber Bleb Nevus Syndrome or BRBNS
Blue Rubber Bleb Nevus Syndrome can be found in people of different eld groups . Individuals from 2 geezerhood old child to adult can be diagnosed with it from all over the world . No cause as such has been found so that the real base cause of the problem can be understand . Both male person and females can be affected by Blue Rubber Bleb Nevus Syndrome or BRBNS .
Diagnosis of Blue Rubber Bleb Nevus Syndrome or BRBNS
The diagnosis of Blue Rubber Bleb Nevus Syndrome or BRBNS can be done through physical examination in most of the cases . However , as and when command the GI tract is checked with the helper of endoscopy . More research is being conduct in this domain so that the accurate drive of the job can be understood well . This will help the doctors to treat and heal their patients well in the future .
Treatment of Blue Rubber Bleb Nevus Syndrome or BRBNS
The treatment that is required for the condition of Blue Rubber Bleb Nevus Syndrome or BRBNS is two faceted where on one side the iron therapy is done to start the development of Fe in the consistency . On the other hand , the removal of the lesions is aim care of . In the first cases where iron needs to be replaced in the organic structure in wicked cases , rakehell transfusion is used for the patients ( 3 ) . When it comes to slay the lesions on the body dissimilar methods can be used like optical maser therapy , lotion of chemicals to injections that will guide to the natural collapse of the wound or a proper surgical remotion . If the lesions are inside the bowel then remove the lesions is never the first option . This is done only when it is prolonged and continued hemorrhage and continual origin transfusions are required by the patient due to the lesions . However , this can be a extended and rather a complex operative process . In many case , such surgical subprogram have been bitter sentence as these lesions can recur even after they have been remove from the system successfully the first time . Again there is another set of opinion bearer who posit that congenital misshapenness should not recur after they have been removed from the gut . This is why the mathematical process has been stated to be one of the most effective way through which Gb bleeding lead out of Blue Rubber Bleb Nevus Syndrome or BRBNS can be regale .
In certain consideration , it has been seen that an program of Argon blood plasma clotting and that of octreotide acetate can be given to the patient suffering from Blue Rubber Bleb Nevus Syndrome and experience Gb bleeding . However , this process can offer impermanent relief and not terminate bleed for a extended fourth dimension . In type the bleeding becomes too severe , it can also wrick out to be a calamitous condition(1 ) .
Conclusion
The lesions in Blue Rubber Bleb Nevus Syndrome or BRBNS are quite a special type of venous malformation . They go on to be modest , multifocal and circumscribed . They could be found in any part of the torso but they are seen in plenty just below the cutis and near the gastrointestinal tracts . The nature of hemorrhage that go on out of these wound is continuous and slow rather than being sudden and really serious at any given point . The literature that has been amass on this experimental condition is only out of the case study that have been done on the stipulation .
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