Attachment in close relationships and glycemic outcomes in children with type 1 diabetes.

BACKGROUND: Our aim was to determine whether child attachment to parents, parent attachment style, and morning cortisol levels were related to diabetes outcomes measured by average glycated hemoglobin (HbA1c), HbA1c variability over 4 years and time in range (TIR) in children with type 1 diabetes (T1D). RESEARCH DESIGN AND METHODS: 101 children with T1D and one of their parents were assessed at baseline for child attachment (Child Attachment Interview; CAI) and parent attachment (Relationship Structures Questionnaire; ECR-RS). Serum samples were collected for cortisol measurements before the interviews. HbA1c levels were measured during a 4-year follow-up period at regular 3-monthly visits, and data for TIR were exported from blood glucose measuring devices. Multivariate linear regression models were constructed to identify independent predictors of glycemic outcomes. RESULTS: More girls than boys exhibited secure attachment to their mothers. The results of the regression models showed that securely attached girls (CAI) had higher average HbA1c than did insecurely attached girls (B = -0.64, p = 0.03). In boys, the more insecure the parent's attachment style, the worse the child's glycemic outcome: the higher the average Hb1Ac (B = 0.51, p = 0.005), the higher the HbA1c variability (B = 0.017, p = 0.011), and the lower the TIR (B = -8.543, p = 0.002). CONCLUSIONS: Attachment in close relationships is associated with glycemic outcomes in children with T1D, and we observed significant differences between sexes. A sex- and attachment-specific approach is recommended when treating children with less favorable glycemic outcomes. Special attention and tailored support should be offered to securely attached girls in transferring responsibility for diabetes care and at least to male children of insecurely attached parents to prevent suboptimal glycemic control. Further studies in larger samples and more daily cortisol measurements may help us better understand the links between stress response, attachment and T1D.

Patient reported outcome measures in children and adolescents with type 1 diabetes using advanced hybrid closed loop insulin delivery.

Purpose: To determine the impact of advanced hybrid closed - loop (AHCL) insulin delivery on quality of life, metabolic control and time in range (TIR) in youth with type 1 diabetes mellitus (T1DM). Methods: Twenty-four children and adolescents with T1DM (14 female) aged of 10 to 18 years participated in the study. Mixed methods study design was implemented. Quantitative part of the study was conducted as a longitudinal crossover study with data collection before and at the end of AHCL use. Qualitative data were obtained with modeled interviews of four focus groups before and the end of the period. Clinical data were collected from the electronic medical records. Results: The use of AHCL significantly improved the quality of life in terms of decreased fear of hypoglycemia (p<0.001), decrease in diabetes-related emotional distress (p<0.001), and increased wellbeing (p=0.003). The mean A1C decreased from 8.55 ± 1.34% (69.9 ± 12.3 mmol/mol) to 7.73 ± 0.42 (61.1 ± 2.2 mmol/mol) (p=0.002) at the end of the study. Mean TIR was 68.22% (± 13.89) before and 78.26 (± 6.29) % (p<0.001) at the end of the study. Conclusion: The use of advanced hybrid closed loop significantly improved the quality of life and metabolic control in children and adolescents with T1DM.

Clinical and genetic characteristics of two patients with tyrosinemia type 1 in Slovenia - A novel fumarylacetoacetate hydrolase (FAH) intronic disease-causing variant.

Tyrosinemia type 1 (HT1) is an inborn error of tyrosine catabolism that leads to severe liver, kidney, and neurological dysfunction. Newborn screening (NBS) can enable a timely diagnosis and early initiation of treatment. We presented the follow up of the only two Slovenian patients diagnosed with HT1. Metabolic control was monitored by measuring tyrosine, phenylalanine and succinylacetone from dried blood spots (DBSs). Retrograde screening of HT1 was performed from DBSs taken at birth using tandem mass spectrometry. First patient was diagnosed at the age of 6 months in the asymptomatic phase due to an abnormal liver echogenicity, the other presented at 2.5 months with an acute liver failure and needed a liver transplantation. The first was a compound heterozygote for a novel FAH intronic variant c.607-21A>G and c.192G>T whereas the second was homozygous for c.192G>T. At the non-transplanted patient, 66% of tyrosine and 79% of phenylalanine measurements were in strict reference ranges of 200-400 µmol/L and >30 µmol/L, respectively, which resulted in a favorable cognitive outcome at 3.6 years. On retrograde screening, both patients had elevated SA levels; on the other hand, tyrosine was elevated only at one. We showed that non-coding regions should be analyzed when clinical and biochemical markers are characteristic of HT1. DBSs represent a convenient sample type for frequent amino acid monitoring. Retrograde diagnosis of HT1 was possible after more than three years of birth with SA as a primary marker, complemented by tyrosine.

Reye Syndrome with Severe Hyperammonemia and a Good Neurological Outcome.

BACKGROUND Reye syndrome (RS) is a rare life-threatening condition combining acute noninflammatory encephalopathy and acute liver failure with an absence of defined etiology. We present a case of fulminant RS that had a good neurological outcome. CASE REPORT A 4-year-old previously healthy boy had no history of acetylsalicylic acid (ASA) use, nor had he been diagnosed with any inborn errors of metabolism. RS was preceded by a mild viral infection, possibly caused by human bocavirus, which has not been previously implicated in RS. He presented with a combination of a very high concentration of ammonia but only mildly elevated aminotransferases and mild hypoglycemia. Computed tomography (CT) of the head additionally showed diffuse cerebral edema with tentorial herniation. The extensive metabolic evaluation did not confirm any inborn errors of metabolism to explain the etiology. We provided optimal treatment of severe hyperammonemia (>500 µmol/L) and cerebral edema, including high doses of arginine chloride, sodium benzoate, hemodialysis, mild hypothermia, and supportive care. He has been followed up for over 4 years. The patient recovered completely, with no long-term psycho-cognitive or neurological sequelae. CONCLUSIONS Although extremely rare, hyperammonemia and RS should be considered in cases of an acute encephalopathy to be treated as soon and as decisively as possible to enable a good outcome.

Validation of the Lifetime Incidence of Traumatic Events (LITE-S/P) Questionnaires in Children and Adolescents in Slovenia.

Introduction: A traumatic event is an extremely threatening and frightening experience in an individual's life. Children who are exposed to traumatic events are twice as likely to develop a mental disorder. Screening can provide insight into the traumatic experience of children, identifying those eligible for further evaluation, and support. With this aim, we evaluated the psychometric properties of the Lifetime Incidence of Traumatic Events questionnaire (LITE) in Slovene by calculating retest reliabilty, construct validity (cross-informant agreement) and external validity, where we calculated the correlation of the number of differenet traumatic events with psychopathological symptoms. Methods: 280 child-parent pairs (children aged 11.3 ± 2.2 years) from various Slovenian primary schools participated in the study. They were divided into two groups: 180 healthy primary school students and 100 children with Type 1 Diabetes (our study was a part of a larger study The Influence of Psychobiological Adversity on Children and Adolescents with Type 1 Diabetes Study). Two versions of the LITE questionnaire were used. Children completed the child report (LITE-S) and parents the parent report (LITE-P) version. After 4 weeks, 117 children, and 114 parents filled out the LITEs again. External validity was assessed using the Youth Self Report and Child Behaviour Checklist syndrome-oriented scales. Results: Retest reliability for individual scales was r = 0.469-0.639 (ρ = 0.443-0.636; p < 0.001), but higher for individual items (κ = 0.263-0.821; p < 0.001). Correlations between reports from parents and children were r = 0.313-0.345 (ρ = 0.317-0.348; p < 0.001). The number of different events experienced by children correlated significantly with the measured depressive-anxiety, and posttraumatic stress disorder symptoms. Conclusions: Based on our results, the LITE-S and LITE-P "All events" scale have acceptable psychometric properties for use in research and in clinical practise screening. We recommend looking at single items, taking into consideration the responses from both the child and the parent for more precise information. To improve the precision of the psychodiagnostic capacity of the questionnaire, further research on various populations should be performed.

Therapy-type related long-term outcomes in mucopolysaccaridosis type II (Hunter syndrome) - Case series.

Mucopolysaccharidosis type II (MPS II, Hunter syndrome) is a rare, X-linked recessive multisystem lysosomal storage disease due to iduronate-2-sulfatase enzyme deficiency. We presented three unrelated Slovenian patients with the severe form of MPS II that received three different management approaches: natural course of the disease without received specific treatment, enzyme replacement therapy (ERT), and hematopoietic stem cell transplantation (HSCT). The decision on the management depended on disease severity, degree of cognitive impairment, and parent's informed decision. The current benefits of MPS II treatments are limited. The lifelong costly intravenous ERT brings significant benefits but the patients with severe phenotypes and neurological involvement progress to cognitive decline and disability regardless of ERT, as demonstrated in published reviews and our case series. The patient after HSCT was the only one of the three cases reported to show a slowly progressing cognitive development. The type of information from the case series is insufficient for generalized conclusions, but with advanced myeloablative conditioning, HSCT may be a preferred treatment option in early diagnosed MPS II patients with the severe form of the disease and low disease burden at the time of presentation.

Psychological Outcomes and Predictors of Weight Loss in Adolescents With Severe Obesity Following a Reversible Endoscopic Bariatric Procedure.

Introduction: Adolescent and children obesity is a growing concern worldwide. Bariatric surgery is used as an effective treatment for adolescents with obesity and provides physical and mental health benefits. Application of alternative, minimally invasive, safe, and reversible endoscopic procedures, such as the Duodenojejunal bypass liner (DJBL), has been recently suggested as an effective treatment for adolescents with obesity. We explored specific psychological outcomes of adolescents with obesity during a year of follow-up after undergoing a reversible endoscopic bariatric procedure, and a year after removal. We were also interested in identifying psychological factors that could predict successful weight loss after the procedure. Methods: Nineteen adolescent with severe obesity undergoing DJBL procedure were psychologically assessed in an open-label, prospective clinical trial (NTC0218393), at the implantation of device and at the removal of device after 12 months. Control group of 26 adolescents with severe obesity were recruited from the same outpatient clinic undergoing only conservative treatment. In addition, adolescents from the intervention group were followed for 12 months after the removal of the device. The Youth Self Report (YSR) was used to assess adolescents' emotional and behavioural problems; The Multidimensional Body-Self Relations Questionnaire (MBSRQ) to assess body image and The Eating Disorder Examination Questionnaire (EDE-Q) to assess attitudes and behaviours related to eating disorder. Results: Significant improvements in somatic complain (F = 12.478, p = 0.001), emotional and behavioural problems (F = 7.169, p = 0.011) and food restraining (F = 9.605, p = 0.004) were found in the intervention group at device removal compared to the control group. Moreover, at the time of device removal compared to baseline, improvements in several psychological outcomes were found (F = 32.178 p = 0.000 for emotional and behavioural problems). Adolescents also became more satisfied with their appearance (F = 6.789, p = 0.019). Majority of observed changes remained stable at the next follow up a year after the device removal. Significant predictors of successful weight loss at device removal were fewer overeating episodes (B = 0.147, p = 0.022) and lower body satisfaction (B = 0.932, p = 0.013). Discussion: Following a reversible bariatric procedure, improvements of psychological (emotional and behavioural) factors were found in adolescents with severe obesity. Psychological predictors of successful weight loss were identified, showing the greatest importance of eating behaviour and body satisfaction in successful weight loss.

Carer's Attachment Anxiety, Stressful Life-Events and the Risk of Childhood-Onset Type 1 Diabetes.

Background/Objective: Type 1 diabetes (T1D) is among the most common chronic diseases in children/adolescents, and the incidence continues to rise worldwide. Different environmental factors have been evaluated in the etiology. In the present study, we investigated the role of attachment examining whether insecure attachment to carers or carers' own attachment insecurity was related to a higher risk of T1D in children. Methods: We included 101 children with T1D (mean age 11.8 years), 106 healthy controls (11.6 years), and one of their carers. We assessed children's attachment using the Child Attachment Interview and carers' attachment using the Relationship Structures Questionnaire. We constructed binary multinomial logistic regression models using attachment to mothers, carers' attachment representations, and stressful life-events as T1D predictors. Results: Higher carer attachment anxiety was associated with the child's T1D diagnosis (p < 0.05; R 2 = 0.0613) while security of attachment to mothers showed no significant association. When mothers' education was included in the model, both attachment anxiety in higher educated mothers and stressful life events showed a significant association with the child's T1D (p < 0.001; R 2 = 0.293). Conclusions: Our findings suggest that higher attachment-related anxiety in carers with high education and stressful life events are associated with T1D in children.

Impact of attention deficit hyperactivity disorder on metabolic control in adolescents with type1 diabetes.

OBJECTIVE: Diabetes mellitus type 1 (T1D) incidence is increasing in pediatric population. Good metabolic control, measured by glycated hemoglobin (HbA1c), significantly reduces the risk for chronic complications. Comorbid disorders, including attention-deficit hyperactivity disorder (ADHD), may influence glycemic control. To date little is known about the prevalence of ADHD among adolescents with T1D and its influence on diabetes self-management. Therefore, we aimed to identify adolescents with T1D and ADHD and assess the effect of ADHD on metabolic control. METHOD: This cross-sectional case-control study included 101 patients (11-17 years old) with T1D. Development and Well-Being Assessment (DAWBA) questionnaire and subsequent psychiatric clinical examination were used to identify ADHD in a group with T1D. Indicators of metabolic control were collected from available medical documentation for preceding 12 months and compared between the group of patients with T1D and ADHD and the group of T1D patients without ADHD. RESULTS: ADHD was diagnosed in 11.9% adolescents with T1D (12 of 101). We found a statistically significant difference (p = .022) in HbA1c between the two groups - higher in the group with T1D and ADHD (8.4% or 68.3 mmol/mol) than in the group with T1D without ADHD (7.8% or 61.7 mmol/mol). CONCLUSIONS: Almost 12% of adolescents with type 1 diabetes were diagnosed with ADHD and they had poorer glycemic control. Adolescents with T1D and ADHD must be diagnosed early and offered appropriate treatment focused on preventing negative ADHD impact on metabolic control.

Fear of hypoglycemia, anxiety, and subjective well-being in parents of children and adolescents with type 1 diabetes.

This study explored the association between parental fear of hypoglycemia, anxiety, and subjective well-being in parents of children and adolescents with type 1 diabetes. A total of 120 mothers and 79 fathers participated. Mothers' and fathers' fear of hypoglycemia was significantly associated with anxiety and negative affect as well as with worse glycemic control in child. Paired-samples t-test showed that mothers were more involved in diabetes management and reported more fear and anxiety compared to fathers, but they did not differ in worries about hypoglycemia. The findings suggest screening for fear of hypoglycemia and subjective well-being in all parents regardless of whether their child experienced severe hypoglycemia.

Reduced Worries of Hypoglycaemia, High Satisfaction, and Increased Perceived Ease of Use after Experiencing Four Nights of MD-Logic Artificial Pancreas at Home (DREAM4).

AIMS: This study assesses the impact of using an AP-system at home on fear of hypoglycaemia. In addition, satisfaction and acceptance of the new technology are evaluated. METHODS: In a multicentre, multinational study of 75 patients using the MD-Logic AP during four consecutive nights in home setting 59 of them (aged 10-54 years, 54% male, HbA1c 7.89 ± 0.69% [62.72 ± 7.51 mmol/mol], diabetes duration 11.6 ± 8.4 yrs) answered standardized questionnaires (HFS, adapted TAM, and AP satisfaction) before and after using the AP. RESULTS: After experiencing the AP in home setting worries of hypoglycaemia were significantly reduced (before 1.04 ± 0.53 versus after 0.90 ± 0.63; P = 0.017). Perceived ease of use as a measure of acceptance with the AP significantly increased after personal experience (before 4.64 ± 0.94 versus after 5.06 ± 1.09; P = 0.002). The overall satisfaction mean score after using the AP was 3.02 ± 0.54 (range 0-4), demonstrating a high level of satisfaction with this technology. CONCLUSIONS: The four-night home-based experience of using MD Logic AP was associated with reduced worries of hypoglycaemia, high level of satisfaction, and increased perceived ease of use of the new technology in children, adolescents, and adults.

Annual Psychological Screening in Youth and Young Adults with Type 1 Diabetes.

AIM: Youth and young adults with type 1 diabetes are at a great risk for developing depression and diabetes specific distress, therefore, systematic psychological screening is recommended. Routine psychological screening was implemented in Slovene diabetes clinic for children, adolescents and young adults in 2012. One-year results are presented. METHODS: Adolescents and young adults (N = 159, aged 11 - 25 years), attending the obligatory yearly educational outpatient visit at University Children's Hospital, Ljubljana, Slovenia, were examined using questionnaires measuring depression (depression scale from Slovene version of Trauma Symptom Checklist for Children) and diabetes distress (Diabetes Distress Screening Scale). Six additional items were included to assess the fear of hypoglycemia and family support. Socio-demographic and diabetes-related data were collected. Questionnaires were analyzed by a psychologist, and the patients that scored above cut-off point were invited to an individual psychological assessment. RESULTS: Of the sample, 1.3 % reached the threshold for elevated depressive symptoms, and 32.7 % reported significant diabetes distress. The need for psychological support from a specialist was expressed by 5.0 %. There were statistically significant associations between all psychological variables; moreover, better glycemic control was associated with lower diabetes distress and better family support. Nine patients (5.7 %) started with psychological treatment according to the referrals after screening. CONCLUSIONS: The results after one year of psychological screening in Slovene type 1 diabetes population displayed small rates of depression and a large proportion of diabetes distress. Only a small percentage of patients attended the offered individual psychological assessment.