Abstract
BackgroundThe neurofibromatoses (NF) are a group of genetic disorders that interfere negatively with the quality of life (QoL) and influence physical, emotional and social statuses. Studying the effects of neurofibromatoses on various aspects of QoL seems important to implement beneficial strategies in increasing the QoL of NF patients. The aim of this study was to review the literature on quality of life in patients with NF and quantitatively evaluate the effects of Neurofibromatosis on various aspects of quality of life by synthesizing available studies.MethodsThis article is written according to the PRISMA checklist. Different databases including PubMed, Scopus, Google scholar and Web of Science were searched to identify studies that examined QoL of patients with neurofibromatosis. The relevant data obtained from these papers were analyzed by a random-effects model. The heterogeneity of studies was calculated using the I2 index and Egger test was used to determine publication bias. The information was analyzed by R and STATA Ver 14.ResultsTwelve studies were selected as eligible for this research and were included in the final analysis. The number of participants in the study was 7314 individuals containing 910 NF patients (642 NF1 and 268 NF2) and 6404 healthy subjects. The mean scores of sub-scales of QoL were significantly lower in NF patients compared with control except for the scale of cohesion. Family and NF patients had lower quality of life in all aspects of QoL than controls. Also, this meta-analysis shows that NF negatively effects on physical function, bodily pain, mental health, social function and general health. Subgroup analysis showed that NF had negative effects on all sub-scales of QoL if the study was conducted in adults and used a SF-36 questionnaire.ConclusionThis meta-analysis suggests that NF is a broad spectrum disease, affecting physical function, bodily pain, mental health, social function and general health.
Highlights
The neurofibromatoses (NF) are a group of genetic disorders that interfere negatively with the quality of life (QoL) and influence physical, emotional and social statuses
Our study reported that QoL is negatively affected by all subdomain physical functions, bodily pain, mental health, social function and general health in NF patients
We conducted a subgroup analysis according to age group; our meta-analysis showed that QoL in all subdomains has decreased significantly in adult NF patients
Summary
The neurofibromatoses (NF) are a group of genetic disorders that interfere negatively with the quality of life (QoL) and influence physical, emotional and social statuses. Neurofibromatosis is a group of inherited genetic disorders (NF1, NF2, and Schwannomatosis) which affects the peripheral and central nervous system; it predisposes affected individuals to develop tumors in the nerve’s sheath across the body; leads to hearing loss, facial weakness, poor gait; and causes chronic pain [1, 2]. Tumors of the cochlear nerve in this disease can lead to loss of hearing, dizziness and vertigo, significant ataxia, equilibrium disorders, headache, tinnitus, pain and dysfunction of the facial muscles [2, 9, 10]. Patients exhibit this disease during the teenage years or second decade of their life [8]. NF1, NF2 and their various physical, cognitive, and social complications can cause a significant decrease in quality of life of affected individuals [3,4,5,6,7,8,9,10]
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