Volume 6, Issue 2 (Spring 2020)                   JCCNC 2020, 6(2): 135-144 | Back to browse issues page


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Abdi M, Jolfaei M, Ghasemi M, Dinmohammadi M R, Torkmandi H, Khademi E. Fazio-Londe Syndrome and Patient-centered Nursing Care: A Case Report. JCCNC 2020; 6 (2) :135-144
URL: http://jccnc.iums.ac.ir/article-1-265-en.html
1- Department of Emergency and Critical Care, Faculty of Nursing and Midwifery, Zanjan University of Medical Sciences, Zanjan, Iran. , mohammad2016@zums.ac.ir
2- Department of Nursing and Midwifery School, Zanjan University of Medical Sciences, Zanjan, Iran.
3- Department of Critical Care Nursing, Nursing and Midwifery School, Zanjan University of Medical Sciences, Zanjan, Iran.
4- Department of Operating Room, Faculty of Nursing and Midwifery, Zanjan University of Medical Sciences, Zanjan, Iran.
5- Department of Nursing, Maragheh Branch, Islamic Azad University, Maragheh, Iran.
Abstract:   (1989 Views)
Introduction: Fazio-Londe disease is a genetic rare disorder manifesting by acute respiratory distress. This article is a case report of Fazio-Londe syndrome, its comparison with other case reports in the literature, and its patient-centered nursing care. 
Case presentation: In this study, we reported a case with Fazio-Londe syndrome with respiratory distress, bulbar palsy, muscle weakness, and other symptoms. 
Management and outcome: The patient was treated with oral riboflavin (100 mg/day), ribavirin (200 mg/day), and intramuscular Vit-B12 (1000 mg/day). She was managed using a patient-centered nursing care model. 
Discussion: Health care providers should be aware of the new and constant symptoms of this rare disease. A patient-centered nursing care model is suggested to manage the disease.
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● Fazio-Londe disease is a genetic rare disorder manifesting by acute respiratory distress.
● Health care providers should be aware of the new and constant symptoms of this rare disease.
● A patient-centered nursing care model is suggested to manage the disease.
Plain Language Summary 
This article is a case report of Fazio-Londe syndrome, its comparison with other case reports in the literature, and its patient-centered nursing care. The health care providers are recommended to follow the home care of Fazio-Londe syndrome patients and consider that this syndrome may be accompanied by new manifestations, such as Ponto bulbar palsy (difficulty swallowing, weak jaw and facial muscles, progressive loss of speech, and weakening of the tongue), muscle pain, chills, sputum, and cyanosis. Also, the patient-centered nursing care model is suggested for providing care for these patients. 

Type of Study: case report | Subject: Special
Received: 2020/02/2 | Accepted: 2020/03/28 | Published: 2020/05/1

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