Burcu Özbaran

Clinical overview of children with mucopolysaccharidosis type III A and effect of Risperidone treatment on children and their mothers psychological status

Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disorder characterized by progressive mental deterioration and severe behavioral problems. We conducted an open-label, crossover study of the efficacy and safety of Risperidone on behavioral disorder in children with MPS IIIA. A total of 12 patients (5.5+/-2.2 years) with enzymatic diagnosis of MPS IIIA were randomly assigned to receive Risperidone (0.125-2mg/d) for 6 months. The hyperactivity and disruptive behavior level of children before and after treatment was evaluated regarding the scores from Turgay DSM IV Based Child and Adolescent Behavior Disorders Screening and Rating Scale (T-DSM-IV-S). Clinic Global Impression Scale - Severity (CGIS-S) was used for all cases for determining the psychiatric disorder severity. The anxiety and depression levels of mothers before and after treatment were evaluated using Hamilton Anxiety Scale (HAM-A) and Beck Depression Inventory (BDI). The adverse effects were evaluated by monitoring weight, serum prolactin, glucose and lipid levels. The response to the treatment was measured by decrease in values of CGI-S (from 6+/-1.12 to 2.91+/-0.66, p=0.001). According to T-DSM-IV-S scores the best improvement was observed in hyperactivity scores (16.25+/-8.57/11.58+/-7.26, p=0.001), followed by opposition/defiance (6.66+/-5.92/5.08+/-4.88, p=0.032), and conduct disorder scores (1.00+/-1.85/0.41+/-.99, p=0.67). No clinically relevant elevations in weight and serum prolactin, glucose or lipid levels have been documented (p>0.05). There was a significant decrease in anxiety and depression scores of mothers (HAM-A: 20.33+/-8.28/17.91+/-6.89, BDI: 23.58+/-7.14/20.5+/-5.93, p<0.001). To our knowledge, research on the pharmacological treatment of MPS IIIA with Risperidone has not been reported. According to our data, Risperidone appeared to be safe and effective in MPS IIIA patients.

Broader autistic phenotype in parents of children with autism: Autism Spectrum Quotient-Turkish version

The Autism Spectrum Quotient (AQ) is a self‐assessment screening instrument for measuring the degree to which an individual of normal intelligence shows autistic traits. Genetic factors could be responsible for the relatives of individuals with autism exhibiting higher than normal rates of autism‐related impairments, referred to as the ‘broader autism phenotype’ (BAP). The aim of this study was to test whether there is a difference between the parents of autistic and those of typically developing children (TDC) on AQ scores in a Turkish sample.

Social and Emotional Outcomes of Child Sexual Abuse: A Clinical Sample in Turkey

Childhood sexual abuse is a traumatic life event that may cause psychiatric disorders such as posttraumatic stress disorder and depression. During 2003-2004, 20 sexually abused children were referred to the Child and Adolescent Psychiatry Clinic of Ege University in Izmir, Turkey. Two years later, the psychological adjustment of these children (M age = 9.4 years, SD = 3.63 years, range = 5 to 16 years) is evaluated. Semistructured interviews, a form for the sociodemographic characteristics, are used for evaluations by a child psychiatrist who is blind to the first evaluation. It is determined that sexually abused children have more psychiatric disorders within the first year than 2 years later. However, some behavior problems occur at both short-term and long-term follow-ups.

The effect of depression on academic achievement in children with epilepsy

In this cross-sectional study our aim was to evaluate the effect of depression on academic achievement in children with epilepsy and low school performance. Fifty-one children with epilepsy and low school performance were evaluated with the Childrens Depression Inventory (CDI) to measure depressive symptoms. School performance was evaluated with Achenbachs Child Behavior Checklist (CBCL) and the Teacher Report Form (TRF). Children diagnosed with depressive spectrum disorders received medical therapy. All tests were administered in the first interview and repeated at the end of 6 months of therapy. Forty-three children completed the study. The patients were evaluated with DSM-IV diagnostic criteria. Accordingly, 9 (20.9%) children had Major Depressive Disorder (MDD) and 4 (9.3%) had Depressive Disorder, Not Otherwise Specified (DD-NOS). All children with MDD and DD-NOS received antidepressant medication, but only seven of them completed treatment. Posttreatment CDI scores were significantly lower, and TRF scores also improved. Pediatric neurologists should be aware of the possibility of depressive disorders in children with epilepsy, especially in those with low school performance.

Psychiatric evaluation of children and adolescents with left ventricular assist devices.

Objectives To evaluate the psychiatric symptoms of children equipped with a ventricular assist device (VAD) and follow them up for 6 months. With the shortage of donor hearts available for the treatment of end-stage heart failure, VADs have been used to provide temporary treatment until a heart becomes available. VADs provide external sources of power for mechanical circulatory support and are capable of sustaining life over weeks and months. This study provides preliminary details about the psychiatric symptoms and disorders of the first eight children equipped with a VAD in Turkey. Methods Eight pediatric patients who recently underwent VAD implantation, aged 1 to 16 years, were evaluated using the Kiddie Schedule for Affective Disorders and Schizophrenia, Child Behavior Checklist, Children’s Depression Inventory, Beck Depression Inventory, and State-Trait Anxiety Inventory for Children and followed up for 6 months. Results In the first evaluation, five participants had a psychiatric disorder diagnosis. Two patients had adjustment disorder with depressive and anxiety symptoms; one had anxiety disorder, not otherwise specified; and two had major depressive disorder. The anxiety and depressive symptom levels in questionnaires were consistent with psychiatric diagnoses. Two patients had heart transplantation during the follow-up period. Conclusions To determine and treat psychiatric symptoms and disorders at an earlier stage, it is important for children and adolescents with a VAD and those who have undergone heart transplantation to be evaluated by a multidisciplinary consultation liaison team including psychiatrists, psychologists, consultant nurses, and counselors. Abbreviations VAD = ventricular assist device K-SADS = Kiddie Schedule for Affective Disorders and Schizophrenia CBCL = Child Behavior Checklist BDI = Beck Depression Inventory CDI = Children’s Depression Inventory STAI-C = State-Trait Anxiety Inventory for Children MDD = major depressive disorder AD = adjustment disorder PE.I = initial psychiatric evaluation PE.II = second psychiatric evaluation

Depression and anxiety levels of the mothers of children and adolescents with left ventricular assist devices.

Ozbaran B, Kose S, Yagdi T, Engin C, Erermis S, Yazici KU, Noyan A, Ozbaran M. Depression and anxiety levels of the mothers of children and adolescents with left ventricular assist devices.

Psychiatric Approaches for Disorders of Sex Development: Experience of a Multidisciplinary Team

Objective: Disorders of sex development (DSD) are a group of congenital medical conditions that affect life as a whole. In this study, we aimed to reflect the experience of a multidisciplinary team in the clinical/psychiatric follow-up of a group of children and adolescents with DSD. Methods: The study group consisted of 51 patients diagnosed with DSD. The Kiddie-Schedule for Affective Disorders and Schizophrenia, Wechsler Intelligence Scale for Children-Revised, Draw a Person Test and Children’s Apperception Test, and the Clinical Global Impression Scale (CGIS) were used for psychiatric evaluations. Results: The mean age of the patients was 7.8 years (median: 7.8; min: 1.0; max: 18.0). Genetic evaluation showed 46,XX configuration in 15 patients (29.4%) and 46,XY in 35 (68.6%). One patient (2.0%) was diagnosed to have a sex chromosome disorder. Forty patients (78.4%) had no problems with their given gender identity and gender role. Thirty-four (66.7%) patients had normal intellectual capacity. Twenty-eight (54.9%) patients did not have any psychiatric problem. Depression, anxiety disorders, attention deficit/hyperactivity disorder, and adjustment disorders were the common diagnoses. The mean score of symptom severity on CGIS-severity-baseline was 6.15±0.68 and after one year, it was 1.46±0.51 (Z=-3.236 p=0.001). The mean score of CGI–Improvement was 1.23±0.44. Conclusion: It is important to identify and treat the psychiatric disorders encountered in patients with DSD. A psychiatrist needs to be included in the professional team following these patients. Examination and observation results need to be shared by holding periodic team meetings to establish a wholesome point of view for every unique child. Conflict of interest:None declared.

Variation in the Oxytocin Receptor Gene Is Associated With Social Cognition and ADHD

Objective: Children with ADHD show substantial deficits in social cognitive abilities. Oxytocin, mediated through its specific receptor (OXTR), is involved in the regulation of social behavior and social cognition. Method: The entire coding sequence of the human OXT and OXTR genes were sequenced to identify mutations and single nucleotide polymorphisms (SNPs) in 151 children with ADHD (ADHD-combined, n = 51; inattentive subtype, n = 50; ADHD-C plus conduct disorder [CD], n = 50; 11-18 years) and 100 healthy controls. Results: We examined the association of three detected SNPs of OXTR with social cognition deficits. A significant association was shown between the children with ADHD and children with CT/TT genotypes of rs4686302 (χ2 = 3.695; p = .037). ADHD children with CT/TT genotype for the OXTR rs4686302 performed significantly lower on the facial emotion recognition task than those with CC genotype. Conclusion: OXTR rs4686302 polymorphism was shown to be a genetic marker in social cognition deficits in ADHD children.

Risperidone, quetiapine and chlorpromazine may have induced priapism in an adolescent

Priapism is the prolonged, painful erection of penile tissue not accompanied by sexual arousal. Priapism has been established as a rare adverse drug reaction to drugs such as antipsychotics, psychostimulants, antidepressants, and mood stabilizers. Immediate intervention is needed to prevent destructive and irreversible complications, such as erectile dysfunction, disfigurement, inability of the penis to stay erect, and related social/emotional problems. Antipsychotic‐induced priapism may result from the alpha receptor occupancy property of those drugs. We report the case of a 13‐year‐old suffering from attention deficit–hyperactivity disorder plus conduct disorder with priapism related to antipsychotics. Episodes occurred with risperidone plus methylphenidate, quetiapine plus methylphenidate, and chlorpromazine alone.

New era of pediatric ventricular assist devices: Let us go to school

As there is still a shortage of pediatric donor hearts, several techniques have been used to assist pediatric patients to survive until transplantation. VADs provide long‐term support and ability of mobilization for children before a suitable heart becomes available. Several devices such as paracorporeal pumps have been used for this purpose, with acceptable morbidity and mortality rates. However, discharge is not possible, as there is no mobile drive unit for these small‐sized pumps. The possible negative psychosocial impact of long‐term hospitalization, away from home and school, may cause some adjustment problems in the future. In this case series, three pediatric patients that underwent intracorporeal LVAD implantation and returned to school are presented to share clinical experience and also to attract attention to the potential social and psychiatric implications.