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Niemann Pick disease is a genetic condition . Niemann Pick disease is of various type such as Type A , B , and C. All the different types of Niemann Pick disease have different onset with different human death . The disease is characterized by failure to metabolize lipoid lead to its accretion . Almost all the organs get affected by Niemann Pick disease . Various factors set off this term .
How Does Niemann Pick Disease Affect The Body?
Neurological disorder : Niemann Pick disease is characterized by progressive neurologic decadence . In character A Niemann Pick disease , death may occur due to neurological loss.1Various neurologic symptom experienced let in increased sensitivity to touch , brain devolution , and blurred speech . The affected role also live loss of intellectual power and suffers from dementia .
Spleen Disorders : Considerable amount of lipid gets salt away in the spleen leading toenlargement of spleen , a consideration know assplenomegaly . There is a high danger of spleen break especially in patient who are coddle in striking sports .
Liver Diseases : Liver enlargementor hepatomegaly also occurs in Niemann Pick disease . The condition is reformist and may conduce to liver nonstarter . Patient , during the summons , may also suffer from austere hepatic conditions such asascitesand persistentjaundice.2
Dysphagia : Dysphagiais consider as the peril factor for mortality in Niemann Pick disease . Oropharyngeal dysphagia is significantly dangerous as it may chair to food or fluid congestion in the lung that leads topneumonia.3
Gastrointestinal disorderliness : problem in the liver touch the overall GI system . The affected role experiences pitiable appetency , malnutrition , gastric distension , and gastric pain .
Poor Posture : Niemann Pick disease make basal ganglia dysfunction . This disfunction leave in the hapless posture of limbs . This guide to difficulty in coordination , balance , and gait .
Ocular Problems : Niemann Pick disease also get visual dysfunction . The patient experiences handicap in the voluntary eye movement , a stipulation also known as supranuclear gaze palsy .
Bone disorderliness : patient with Niemann Pick disease have unnatural evolution of the bony scheme . patient have short height and the mineralization of osseous tissue is slow.4The bone center dental caries get enlarge and the cortical bone becomes thin .
Muscle Health : Niemann Pick disease have a sudden red of sinew tonus . Gelastic cataplexy is seen in such patients . The patient also experiences a sudden release of muscle tone while walking or standing which may leave to a fall.5
Sleep Disorder : Niemann Pick disease affect various electric cell , especially hypocretin secreting cell , of the hypothalamus . This result in sleep disturbances . The patient may experience quietus anastrophe i.e. the patient role slumber in the day and stay alive during the night .
Triggers For Niemann Pick Disease
There is no cure for Niemann Pick disease . The treatment is take to oversee the symptom of this condition . One of the important scheme adopted in managing the symptom is to keep the patient away from the triggers . Some of the triggers for Niemann Pick disease are :
Emotional Stimulation : Strong aroused foreplay may result in increased stiffness of Niemann Pick disease . People who get angry are at mellow endangerment of aggravate of symptoms .
Physical Exertion : straining activities and forcible travail step-up the severity of Niemann Pick disease symptom .
Unexpected laugh : Cataplexy in Niemann Pick disease can be triggered by unexpected laugh . A affected role with Niemann Pick disease develops “ pearl approach ” during lauchter.6
Conclusion
Niemann Pick disease affects almost every part of the torso . The organs affect most commonly are spleen , liver , brain , bones and gastrointestinal system . The disease cause sleep disturbances , poor intellectual power , poor growth , malnutrition , dysphagia , liver failure , splenomegaly , and dying . Triggers for exacerbate the symptom of Niemann Pick disease are strong emotions , and forcible travail .
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