ABSTRACT
OBJECTIVES: Malignant Infantile Osteopetrosis (MIOP) is a rare inherited disorder with neurological complications, notably visual impairment and decrease of hearing level. Although Hematopoietic Stem Cell Transplantation (HSCT) has been approved as the only curative treatment for these patients, the exact impact of it on visual and hearing level is still unclear. STUDY DESIGN: We analyzed the P2 latency and amplitude from Visual Evoked Potentials (VEP) of 10 patients (20 eyes) and the threshold of wave V from Auditory Brainstem Response (ABR) of 15 patients (30 ears) with MIOP before, 6 and 12 months after HSCT. RESULTS: Before the HSCT, 10/30 ears demonstrated some degree of hearing loss; while only 3/20 eyes had P2 wave latencies in normal range for age. Using GEE models, it was shown that 12 months after HSCT, wave V threshold of ABR of the patients was significantly lower compared to its value from before the transplant (p value: 0.04). The analysis of latency and amplitude of P2 wave of VEPs showed no significant difference between before and after the transplant. CONCLUSION: This study clearly showed that HSCT can improve the hearing level of the patients in terms of ABR threshold. Although HSCT made no significant improvement in latency of P2 in VEP of the patients, it can be concluded that transplant can halt visual regression in these patients. Early diagnosis of MIOP with this objective tools and subsequently early HSCT in these patients can decrease the rate of neurological complications of MIOP and improve the quality of life in them.
Subject(s)
Evoked Potentials, Auditory, Brain Stem/physiology , Evoked Potentials, Visual/physiology , Hematopoietic Stem Cell Transplantation/methods , Osteopetrosis/complications , Osteopetrosis/therapy , Adolescent , Adult , Child , Female , Hearing Loss/etiology , Humans , Male , Middle Aged , Quality of Life , Vision Disorders/etiology , Young AdultABSTRACT
BACKGROUND: Now a days the utilization of Acceptance / Commitment and Emotion Regulation Strategy as a comprehensive treatment plan has been discussed in both the prevention and the control of destructive and risky behaviors. Treatment based on Acceptance/Commitment and Emotion Regulation was effective in both the improvement and the control of high-risk behaviors of students with dyscalculia. OBJECTIVES: The purpose of this study was to investigate the effectiveness of Acceptance and Commitment, and Emotional Regulation training in high-risk behaviors of students with dyscalculia. MATERIALS AND METHODS: This research was experimental, with pre-test, post-test and a control group. The statistical universe of this study included all sixth-grade male students in Ardabil city in the academic year of 2012-2013 (A.H.). The subjects of this study involved 800 sixth-grade elementary students in Ardabil province, selected using a multi-stage cluster sampling. From among them, 60 students with dyscalculia were selected using random sampling method after the initial diagnosis by structured clinical interview and the Keymath Mathematic test. Twenty pupil were selected for either the experimental or the control group. To collect data, the questionnaires of "Keymath Mathematic test" and High-risk Behavior" were used. RESULTS: The results of Multivariate Analysis of Variance (MANOVA) showed that "Acceptance / Commitment and Emotion Regulation" treatment trainings were effective in reducing high-risk behaviors, in a manner that they led to a reduction in negative emotions, self-destructive and impulsive behaviors of students with math disorder (dyscalculia). CONCLUSIONS: It can be concluded that teaching these skills to the students has been influential in enhancing awareness level and change or positive attitude creation in the subjects. Therefore, it is essential to design and implement interventions based on "prevention caused by the peer group, in collaboration with the parents either at the school or at home among the family members".
