Niemann Pick Tip C Hastalığı Olan Bir Çocuğun Hemşirelik Bakımı: Olgu Sunumu
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Dosyalar
Tarih
2016
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Cilt Başlığı
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Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Niemann-Pick hastalığı, vücudun çeşitli organlarında, özellikle retiküloendotelyal sistem ve santral sinir sisteminde, sŞ ngomyelin ve kolesterol birikimi ile karakterize, otozomal resesif geçişli, nadir görülen bir lizozomal lipid depo hastalığıdır. Hastalığın Tip A, B, C, D, E ve F olmak üzere altı değişik formu bulunmaktadır. Klinik belirtiler hastalığın tipine göre değişiklik göstermekle birlikte hepatosplenomegali, tekrarlayan solunum yolu enfeksiyonları, mental retardasyon, büyüme-gelişme bozuklukları, spastisite, göz bulguları, miyoklonik nöbetler ve ataksi gibi hepatik, nörolojik ve psikiyatrik bulgular görülebilmektedir. Bu çalışmada Niemann-Pick Tip C tanısı alan yedi aylık A olgu olarak sunulmuştur. A'nın sağlık öyküsü Marjory Gordon'un "Fonksiyonel Sağlık Örüntüleri"ne göre değerlendirilmiştir. Hemşirelik tanısı olarak solunum fonksiyonunda bozulma (ventilatörden ayrılmaya disfonksiyonel tepki), büyüme ve gelişmede gecikme, korunmada etkisizlik (deri bütünlüğünde bozulma), uyku örüntüsünde bozulma, etkisiz termoregülasyon, bebek davranışının disorganizasyonu, oral müköz membranlarda bozulma riski, aspirasyon riski ve anneye yönelik olarak anksiyete, ümitsizlik, bakım verici rolünde zorlanma koyularak standart hemşirelik girişimleri uygulanmıştır
Niemann-Pick disease is a rare, autosomal recessive lysosomal lipid storage disorder characterized by the accumulation of sphingomyelin and cholesterol in various organs in the body, especially in the reticuloendothelial system and central nervous system. The disease has six different forms including Type A, B, C, D, E and F. With clinical symptoms varying based on the type of the disease, hepatic, neurological and psychiatric Ş ndings such as hepatosplenomegaly, recurrent respiratory tract infections, mental retardation, growthdevelopment disorders, spasticity, vision Ş ndings, myoclonic seizures and ataxia may be observed. Seven-month old A., who was diagnosed with Niemann-Pick Type C and whose health history was assessed based on Marjory Gordon’s “Functional Health Patterns”, is presented as a case study. Standard nursing interventions were performed by making a nursing diagnosis on the child of impaired respiratory function (dysfunctional ventilatory weaning response), delayed growth and development, ineffective protection (impairment in skin integrity), disturbed sleep pattern, ineffective thermoregulation, disorganized infant behaviours, impaired risk in oral mucous membranes, aspiration risk, and by diagnosing the mother with anxiety, hopelessness, and caregiver role strain
Niemann-Pick disease is a rare, autosomal recessive lysosomal lipid storage disorder characterized by the accumulation of sphingomyelin and cholesterol in various organs in the body, especially in the reticuloendothelial system and central nervous system. The disease has six different forms including Type A, B, C, D, E and F. With clinical symptoms varying based on the type of the disease, hepatic, neurological and psychiatric Ş ndings such as hepatosplenomegaly, recurrent respiratory tract infections, mental retardation, growthdevelopment disorders, spasticity, vision Ş ndings, myoclonic seizures and ataxia may be observed. Seven-month old A., who was diagnosed with Niemann-Pick Type C and whose health history was assessed based on Marjory Gordon’s “Functional Health Patterns”, is presented as a case study. Standard nursing interventions were performed by making a nursing diagnosis on the child of impaired respiratory function (dysfunctional ventilatory weaning response), delayed growth and development, ineffective protection (impairment in skin integrity), disturbed sleep pattern, ineffective thermoregulation, disorganized infant behaviours, impaired risk in oral mucous membranes, aspiration risk, and by diagnosing the mother with anxiety, hopelessness, and caregiver role strain
Açıklama
Anahtar Kelimeler
Eğitim, Eğitim Araştırmaları
Kaynak
Hemşirelikte Eğitim ve Araştırma
WoS Q Değeri
Scopus Q Değeri
Cilt
13
Sayı
3
