Ultrastructural Changes in Skeletal Muscle of Infants with Mitochondrial Respiratory Chain Complex I Defects

BACKGROUND AND PURPOSE: The pathogenesis of mitochondrial disease (MD) involves the disruption of cellular energy metabolism, which results from defects in the mitochondrial respiratory chain complex (MRC). We investigated whether infants with MRC I defects showed ultrastructural changes in skeletal muscle. METHODS: Twelve infants were enrolled in this study. They were initially evaluated for unexplained neurodegenerative symptoms, myopathies, or other progressive multiorgan involvement, and underwent muscle biopsies when MD was suspected. Muscle tissue samples were subjected to biochemical enzyme assays and observation by transmission electron microscopy. We compared and analyzed the ultrastructure of skeletal muscle tissues obtained from patients with and without MRC I defects. RESULTS: Biochemical enzyme assays confirmed the presence of MRC I defects in 7 of the 12 patients. Larger mitochondria, lipid droplets, and fused structures between the outer mitochondrial membrane and lipid droplets were observed in the skeletal muscles of patients with MRC I defects. CONCLUSIONS: Mitochondrial functional defects in MRC I disrupt certain activities related to adenosine triphosphate synthesis that produce changes in the skeletal muscle. The ultrastructural changes observed in the infants in this study might serve as unique markers for the detection of MD.

Screening for depression and anxiety disorder in children with headache / 소아과

PURPOSE: The purpose of this study was to investigate the importance of initial screening tests for depression and anxiety disorders in children with headache. In addition, this study evaluated whether the Children's Depression Inventory (CDI) and Revised Children's Manifest Anxiety Scale (RCMAS) are suitable for screening symptoms of depression and anxiety. METHODS: A retrospective chart review was conducted of 720 children aged 7-17 years who had visited a pediatric neurology clinic for headaches and were referred to a pediatric psychiatric clinic for psychiatric symptoms from January 2010 to December 2011. All patients completed the CDI and RCMAS. Among them, charts of patients with clinically significant total scores (cutoff>15) for psychiatric symptoms, as defined by the CDI and RCMAS scoring scales, were reviewed. RESULTS: Nineteen patients had headaches and clinically significant total scores for psychiatric symptoms. The mean age at headache diagnosis was 11.7 years, and 57% were male. Mean duration of headache was 11.5 months. Two point eight percent of the patients were diagnosed with psychiatric disorders including major depression (1.7%), generalized anxiety disorder (1.1%), and bipolar disorder (0.1%). Four patients (0.6%) were diagnosed with attention deficit/hyperactivity disorder (ADHD). Total mean CDI and RCMAS scores of patients referred to the psychiatric clinic were 18.8 and 22.2, respectively. There was no correlation between CDI or RCMAS total scores and headache frequency, duration, or severity. CONCLUSION: We recommend that all patients with headache should be screened for depression and anxiety by CDI and RCMAS scores.

Exploring the Subtypes of Intellectual and Psychological Characteristics in Children with Brain Tumors and Their Mothers' Parenting Stress / 대한소아신경학회지

PURPOSE: Parenting stress could be different among mothers of children with brain tumors. The aim of this study was to explore subtypes of children with brain tumor based on their intellectual and psychological characteristics and to compare the parenting stress of their mothers among the subtypes. METHODS: Latent profile analysis was conducted on the Verbal IQ, Performance IQ, and the scores of Social Problem, Internalization Behavior Problem, and Externalization Behavior Problem in a total of 90 children with brain tumors. Furthermore, the mothers' parenting stress was compared among the explored subtypes. RESULTS: Four-categorization appeared the best way to describe the intellectual and psychological presentation of the children with brain tumors and they are as follows; (1) Borderline Intelligence-Socially Maladjusted (B-SM: 24.4%), (2) Average Intelligence-Internalization Behavior Problem (A-I: 44.4%), (3) Average Intelligence-Normal Behavior (A-N: 18.9%), and (4) Retarded Intelligence-Global Maladjusted (R-GM: 12.2%). Mothers of R-GM group reported higher levels of stress on total domain and the subfactors of distractibility and adaptability of Korean Parenting Stress Index compared to mothers of A-N group, and the highest level of stress on spouse subfactor among the mothers of all subtypes. Mothers of subtypes with either intellectual or psychological problems showed higher level of stress on demandingness subfactor compared to mothers of A-N group. CONCLUSION: Eighty percents of children with brain tumor showed intellectual or psychological problems and the mothers of the children with both problems reported the highest parenting stress. The tailored services are needed to manage the parenting stress of mothers of children with brain tumors.

The Emotional Distress Prior to the Surgery and the Parenting Stress in Elementary School Children with Neurosurgical Diseases: The Preliminary Study

OBJECTIVES: This study was conducted to compare emotional problems in children with neurosurgical diseases prior to surgery as well as levels of parenting stress experienced by mothers of children treated with surgery and those without surgery. The goals was to provide a basis on which comprehensive treatment interventions could be established. METHODS: Subjects included 78 elementary school children who visited the Department of Pediatric Neurosurgery as well as their mothers. The Children's Depression Inventory (CDI) and the Revised Children Manifest Anxiety Scale (RCMAS) were administered to children. The Parenting Stress Indexs (PSI) and Beck Depression Inventory (BDI) were administered to their mothers. RESULTS: The children with neurosurgical diseases showed higher levels of depression prior to surgery. In their mothers, parenting stress was relatively higher and parenting competence was lower. In particular, for those children who underwent surgery, depression was significantly higher. Specifically, they exhibited more negative expectations, and negative self-image. In mothers of children who underwent surgery, depression was significantly higher. CONCLUSION: Our results suggest higher levels of emotional distress and parental stress in children who undergo neurosurgical operations and their mothers, respectively. Based on this, the necessity for surgical and comprehensive psychological intervention is suggested.