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For some people their jewelry is not about style but more about power, class, aggression Roquan Smith Balck Jersey , rage, rebellion and attitude. Skull jewelry have to be a mix of all these elements to make the wearers complete justice and showcase their persona with full confidence.
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鈥?Described by 鈥淲illiam an Beuren鈥?in 1961
鈥?Rare genetic Condition
鈥?The clinical manifestations include a distinct facial appearance, cardiovascular anomalies that may be present at birth or may develop later in life, idiopathic hyper calcemia and a characteristic developmental and behavioral profile.
Path physiology:-
Haploinsufficiency due to a deletion at chromosome band 7q11.23 that involves the elastin gene (ELN) is implicated.
鈥?William syndrome is not solely caused by elastin haploinsufficiency; the deletion involves a region that spans more than 28 genes 鈥?Contiguous gene deletion syndrome.
鈥?Copy number variants in the 7q11.23 region have been found to be associated with autism in a study of over 4000 individuals who did not have William syndrome.
Epidemiology:-
鈥?US 鈥?17500 -20 Khalil Mack Balck Jersey ,000 birth
鈥?Mortality 鈥?Cardiovascular disease
鈥?Sudden Death 鈥?SVAS, Severe pulmonary stenosis and myocardial ischemia secondary to either coronary insufficiency or biventricular outflow tract obstruction with ventricular hypertrophy.
鈥?Hypertension - 50%
鈥?Renal artery stenosis
鈥?A higher frequency of obesity, impaired glucose tolerance and diabetes mellitus have been found in adults with William syndrome compared to the general population
鈥?Increased TSH. Increased prevence in points with William syndrome.
鈥?GI problems 鈥?feeding problems and colic , reflox and chronic constipation
鈥?Sigmoid diverticolitis in adults more in William syndrome.
鈥?It is a multisystem condition with other potential consequences including developmental delay Walter Payton Balck Jersey , motor delay, hearing loss, severe dental disease, ocular problems Authentic Riley Ridley Jersey , progressive joint contractures, nephrolithiasis, bowel and bladder diverticula.
Gender:-MF
鈥?A greater severity and earlier presentation of cardiovascular disease may be observed in males.
Age:-Birth to adulthood
鈥?Features that may be detected antenatally include the characteristics cardiovascular lesions.
鈥?Fetal ultrasonography of neonates with William syndrome has revealed multicystic dyplastic kidney in addition to congenital heart lesions.
鈥?Associated findings on prenatal screening that have been reported include an increased fetal nuchal transluency and low maternal serum alpha fetoprotein (MSAFP)
Clinical Presentation:-
It includes a pattern of growth and development and a specific neurodevelopment profile primarily involving 4 areas:-
1. Cognitive Development
2. Language
3. Auditory function
4. VIsuospatial function
鈥?Children have prenatal and postnatal growth delay and usually present with failure to thrive
鈥?Short stature
鈥?Poor weight gain and feeding problems
鈥?Recurrent middle ear infections.
鈥?Visual disturbances 鈥?mainly related to esotropia, cataracts and hyperopia in as many. Wholesale Nike Shoes Cheap   Wholesale Air Jordan Cheap   Wholesale Air Max Cheap   Wholesale Nike Shoes   Cheap Jordan Shoes   Cheap Nike Shoes China   Wholesale Nike Air Max   Wholesale Max Shoes   Wholesale Nike Shoes Shop   Wholesale Nike Shoes Online
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