Cystic fibrosis related diabetes (CFRD) is a comorbidity of cystic fibrosis (CF) that negatively impacts on its clinical course. Prediabetes is an important predictor of either CFRD development and unfavorable prognosis of CF in both pediatric and adult patients. International guidelines recommend insulin only in case of CFRD diagnosis. Whether early detection and treatment of prediabetes may contribute to improve the clinical course of CF is still debated. A subgroup of pediatric diabetologists of the Italian Society for Pediatric Endocrinology and Diabetology (ISPED) performed a systematic review of the literature based on predefined outcomes: impact of pre-diabetes on clinical outcomes and on the risk of developing CFRD; diagnosis of diabetes and pre-diabetes under 10 years of age; effectiveness of therapy on glycemic control, impact of therapy on pulmonary function and nutritional status. Thirty-one papers were selected for the analysis data presented in these papers were reported in tables sorted by outcomes, including comprehensive evidence grading according to the GRADE approach. Following the grading of the quality of the evidence, the entire ISPED diabetes study group achieved consensus for the Italian recommendations based on both evidence and clinical experience. We concluded that in patients with CF, prediabetes should be carefully considered as it can evolve into CFRD. In patients with CF and prediabetic conditions, after complete evaluation of the OGTT trend, glucometrics, glycemic values measured during pulmonary exacerbations and/or steroid therapy, early initiation of insulin therapy could have beneficial effects on clinical outcomes of patients with CF and prediabetes.
Diagnosis of type 1 diabetes (T1D) in a child is often associated with anger, denial, fear, and depression from the parents. The aim of the study was to improve parents' adaptation to the diagnosis of diabetes of their child. Sixty-two parents (29 mothers, 33 fathers) of 36 children with type 1 diabetes (mean age = 11.3‐3.3 years; diabetes duration > 1 year; HbA1c = 57 ± 11 mmol/mol) participated in a three-day educational working group pilot intervention study. Intervention was based on the reexamination of the traumatic event of diagnosis of T1D through spatial and time-line anchorage, retracing of the future, emotional awareness, and interactive discussion. Relaxing technique, diaphragmatic breathing, and guided visualization were used by 2 psychologists and 1 pediatric endocrinologist. The study was approved by EC and participants filled a consent form. At baseline and after intervention, parents filled in a questionnaire including Diabetes-Related Distress (DRD), Parent Health Locus of Control Scale (PHLOC), Parent Stress Index Short Form (PSI-SF), Hypoglycemia Fear Survey-Parents (HFS-P) and Hypoglycemia Fear Survey-Parents of Young Children (HFS-P-YC), and Health Survey Short Form-36 (SF-36). Three months after the intervention, both parents reported a reduction in the “difficult child” subscale of the PSI-SF (p < 0.05) and increased scores of social functioning of the SF-36 (p < 0.05). DRD score was significantly reduced in mothers (p = 0.03), while the “parental distress” subscale of the PSI-SF was significantly improved in fathers (p = 0.03). This weekend-based parent group intervention seems to reduce stress and improve social functioning of parents of children and adolescents with type 1 diabetes.
Background Glycogen storage disease type XII is a rare metabolic disease resulting from Aldolase A deficiency that causes muscle glycogen accumulation, with crisis of rhabdomyolysis and hemolytic anemia. In the very few cases described, rhabdomyolysis crises are caused by fever and/or exercise and can accompany acute hemolytic anemia. Although currently there is no therapy available for this disease, the guidelines for the management of other forms of glycogen storage diseases recommend a nutritional therapy in order to avoid hypoglycemia or prevent exercise-induced rhabdomyolysis. Case presentation In this case report we describe a new phenotype of the disease in a 14-year-old boy, characterized by seizures and rhabdomyolysis. Beside an antiepileptic treatment, we propose a new therapeutic approach based on ketogenic diet in order to supply an energetic substrate for skeletal muscle and neurons. Conclusions The anti-epileptic therapy and the dietetic approach were well tolerated by the patient who showed good compliance. This led to a deceleration of the disease with no other acute episodes of seizures and rhabdomyolysis, without any side effects observed.
Aim of this study was to evaluate the association of socio-demographic and clinical factors, including technologies used for diabetes management, and parental fear of hypoglycaemia (FOH) in parents of children (<18y) with T1D. A cross-sectional, nationwide electronic survey, supported by the Italian Society of Paediatric Endocrinology and Family Associations of children with diabetes, was conducted during September-November 2018. The Hypoglycaemia Fear Survey for Parents (HFS-P), a 25-item questionnaire, was used to assess FOH. Quintile regression analysis was performed to estimate factors associated to FOH. Out of 1450 questionnaires, 1106 (873 mothers) reported a complete HFS-P in both the worry and behavior subscales. Children median age was 11 y (IQR: 8-14), median diabetes duration 6 y (IQR: 3-10); median HbA1c 7.2% (IQR: 6.7-7.8). Glucose Sensor (including CGM and FGM) was used in 79% of patients and insulin pump in 45%. Median HFS-P worry score was 36 (IQR: 29-46), behavior score 29 (IQR: 24-33). Parental FOH was associated with parental modifiable and non-modifiable factors (Table 1). The use of insulin pump does not seem to impact worries and behaviors, while glucose sensor do influence parental behaviors. As the use of diabetes-advanced technologies is today increasingly widespread, the impact on parental FOH requires more analyses. Disclosure V. Cherubini: None. D. Pjetraj: None. R. Bonfanti: Advisory Panel; Self; Abbott, Lilly Diabetes, Medtronic, Novo Nordisk A/S, Roche Diabetes Care, Sanofi. M. Marino: None. A. Iannilli: None. F. Carle: None. R. Gesuita: None.
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