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1.
Diabet Med ; : e15333, 2024 Apr 26.
Artículo en Inglés | MEDLINE | ID: mdl-38671595

RESUMEN

AIMS: Time in Tight Range (TITR) is a novel glycaemic metric in monitoring type 1 diabetes (T1D) management. The aim of this study was to assess the attainability of the TITR target in children and adolescents using the advanced hybrid closed loop (AHCL). METHODS: The 2128-day CGM data from 56 children and adolescents with T1D using AHCL (Minimed-780G) were analysed. Time in Range (TIR) (3.9-10 mmol/L), TITR (3.9-7.7 mmol/L), and other glycaemic parameters were separately analysed in terms of whole day, daytime (06.00-23:59), and nighttime (00.00-05.59) results. The participants were divided into two groups by autocorrection rate where Group 1 had a rate of <30% and Group 2 had a rate of ≥30. RESULTS: All glycaemic parameters indicated a better glycaemic outcome in the nighttime with higher TIR and TITR values compared with daytime (for TIR 87.5 ± 9.5% vs. 78.8 ± 8%, p < 0.001, and TITR 68.2 ± 13.5% vs. 57.5 ± 8.8%, p < 0.001). The rates of TITR >50% and >60% were 87% and 52%, respectively. When those with TITR >60% (n: 29) and those without (n: 27) were evaluated in terms of hypoglycaemia, no statistically significant difference was found in time below range (TBR) 3-3.9 mmol/L (0.3% vs. 2.1%, p: 0.084) and TBR < 3 mmol/L (0.47% vs. 0.3%, p: 0.298). Group 1 had a significantly higher TIR and TITR compared to Group 2 (82.6 ± 6.1% vs. 75.6 ± 8.6%, p: 0.008 and 62.1 ± 7.5% vs. 53.8 ± 7.5%, p: 0.002, respectively). CONCLUSIONS: Most children and adolescents on AHCL achieved the 50% target for TITR whereas more than half achieved the >60% target. A target of >50% for TITR seems realistic in children with T1D using AHCL.

2.
BMC Endocr Disord ; 21(1): 18, 2021 Jan 23.
Artículo en Inglés | MEDLINE | ID: mdl-33485357

RESUMEN

BACKGROUND: Currently, there is a lack of data relating to glycemic parameters and their relationship with C-peptide (CP) and proinsulin (PI) during the partial remission period (PRP) in type 1 diabetes mellitus (T1D). The aim of this study was to evaluate glycemic parameters in children with T1D who are in the PRP using intermittently scanned continuous glucose monitoring systems (isCGMS) and to investigate any relationships between CP and PI levels. METHODS: The study included 21 children who were in the PRP and 31 children who were not. A cross-sectional, non-randomized study was performed. Demographic, clinical data were collected and 2 week- isCGMS data were retrieved. RESULTS: The Serum CP showed a positive correlation with time-in-range in the PRP (p:0.03), however PI showed no correlations with glycemic parameters in both periods. The Serum CP and PI levels and the PI:CP ratio were significantly higher in the PRP group than in the non-PRP group. In the non-PRP group, the PI level was below 0.1 pmol/L (which is the detectable limit) in only 2 of the 17 cases as compared with none in the PRP group. Similarly, only 2 of the 17 children in the non-PRP group had CP levels of less than 0.2 nmol / L, although both had detectable PI levels. Overall time-in-range (3. 9-1.0 mmol/L) was significantly high in the PRP group. In contrast, the mean sensor glucose levels, time spent in hyperglycemia, and coefficient of variation levels (32.2vs 40.5%) were significantly lower in the PRP group. CONCLUSIONS: Although the mean glucose and time in range during the PRP was better than that in the non-PRP group, the glycemic variability during this period was not as low as expected. While the CP levels showed an association with TIR during the PRP, there was no correlation between PI levels and glycemic parameters. Further studies are needed to determine if PI might prove to be a useful parameter in clinical follow-up.


Asunto(s)
Péptido C/sangre , Diabetes Mellitus Tipo 1/sangre , Proinsulina/sangre , Adolescente , Glucemia , Automonitorización de la Glucosa Sanguínea , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Hiperglucemia/sangre , Hipoglucemiantes/uso terapéutico , Insulina/uso terapéutico , Masculino , Remisión Espontánea
5.
Horm Res Paediatr ; 97(2): 172-179, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-36972563

RESUMEN

INTRODUCTION: Endogenous Cushing's syndrome (CS) is a rare, severe disease that can cause multiple systemic involvements and behavioral problems due to excessive cortisol production. Structural changes can be noted in the brain magnetic resonance imaging (MRI) scans of these cases. CASES: A 9-year-old girl and a 13-year-old boy were admitted with hypercortisolism. In the female patient, altered consciousness was prominent along with cerebral and cerebellar brain atrophy, and findings indicating posterior reversible encephalopathy syndrome were detected in the brain MRI. Although the male patient's neurological examination was normal, significant cerebral atrophy was seen in the brain MRI. Case 1 was diagnosed as having ectopic ACTH syndrome (EAS) due to a thymic carcinoid tumor. Case 2 underwent a pulmonary lobectomy upon detection of a bronchial lesion in the Ga-68 DOTATATE PET/CT scan while being examined for EAS due to a lack of suppression in the high-dose dexamethasone suppression test. However, hypercortisolism persisted despite the removal of the bronchial lesion, and subsequently, a diagnosis of Cushing's disease was established following bilateral inferior petrosal sinus sampling. DISCUSSION: Endogenous hypercortisolism may cause brain atrophy of varying severity. The central nervous system findings can be overlooked in children with CS. More comprehensive studies are needed to better understand the behavioral changes caused by the effects on the brain and to evaluate whether these changes are reversible. In addition, identifying the source of hypercortisolism can be difficult due to a lack of experience related to the rarity of the disease in children.


Asunto(s)
Síndrome de ACTH Ectópico , Síndrome de Cushing , Síndrome de Leucoencefalopatía Posterior , Humanos , Masculino , Femenino , Niño , Adolescente , Síndrome de Cushing/diagnóstico por imagen , Síndrome de Cushing/etiología , Radioisótopos de Galio , Tomografía Computarizada por Tomografía de Emisión de Positrones/efectos adversos , Síndrome de Leucoencefalopatía Posterior/complicaciones , Síndrome de ACTH Ectópico/diagnóstico , Síndrome de ACTH Ectópico/etiología , Atrofia/complicaciones
6.
Artículo en Inglés | MEDLINE | ID: mdl-38488051

RESUMEN

Successful management of type 1 diabetes (T1D) requires not only optimal glycemic outcomes, but also a holistic approach that encompasses all aspects of life and recommendations to address needs. Current goals include optimal glycaemic values, quality of life and life expectancy similar to peers, prevention of long-term complications, prevention of severe hypoglycaemia as much as possible, facilitation of glucose management, etc. International Society for Pediatric and Adolescent Diabetes (ISPAD) has been updating its guidelines for diabetes care every 4 years since 1995, covering more and more topics. For optimal metabolic outcomes, diabetes teams need to follow these current recommendations, adapt them to their clinical practice and provide guidance to people with type 1 diabetes/families. In this review, in the light of ISPAD 2018-2022 guidelines and clinical experiences, "10 Key Recommendations", emphasizing the importance of teamwork and the use of technology, current type 1 diabetes treatment is described for practical applications.

7.
Artículo en Inglés | MEDLINE | ID: mdl-38953734

RESUMEN

Objective: To determine inequalities in access to diabetes technologies and the effect of socioeconomic factors on families with children with type 1 diabetes. Methods: In this multicenter cross-sectional study, parents of children with type 1 diabetes completed a questionnaire about household sociodemographic characteristics, latest HbA1c values, continuous glucose monitoring (CGM) and insulin pump use of children, the education and working status of parents. These characteristics were compared between technology use (only-CGM, only-pump, CGM+pump, no technology use). Results: Among 882 families, only-CGM users, only-pump users, and CGM+pump users compared with no technology users, adjusting for age, sex, region, education levels, number of working parents, and household income. Children living in the least developed region had lower odds of having only-CGM (OR=0.20, 95%CI 0.12-0.34) and having CGM+pump (OR=0.07, 95%CI 0.03-0.22) compared with those living in the most developed region. Children with parents who had not finished high school had lower odds of having only-CGM (Mothers: OR=0.36, 95%CI 0.19-0.66; fathers: OR=0.32, 95%CI 0.18-0.60) or both CGM+pump (OR=0.27, 95%CI 0.11-0.64; fathers: OR=0.34, 95%CI 0.15-0.79) rather than no-technology compared to children whose parents has a university degree. Every $840 increase in the household income increased the odds by 5% for having only-CGM (OR=1.05, 95%CI 1.02-1.09) and CGM+pump (OR=1.05, 95%CI 1.01-1.08). Conclusion: Socioeconomic factors such as education, regions, and income were associated with inequality in access to technologies. The inequalities are more prominent in access to CGM while CGM had a bigger contribution to glycemic control.

8.
Artículo en Inglés | MEDLINE | ID: mdl-38664994

RESUMEN

Introduction: Craniopharyngiomas (CPG) have complex challenges in treatment due to their proximity to vital structures, surgical and radiotherapeutic complexities, and the tendency for recurrence. This study aims to identify the prevalence of endocrine and metabolic comorbidities observed during initial diagnosis and long-term follow-up in a nationwide cohort of pediatric CPG patients. The study also highlights the associated difficulties in their management. Methods: Sixteen centers entered 152 patients into the ÇEDD NET data system. We evaluated the clinical and laboratory characteristics at presentation, administered treatments, accompanying endocrine, metabolic, and other system involvements, and the patient's follow-up features. Results: Of the evaluated patients, 64 were female, and 88 were male. At presentation, the mean age was 9.1 ± 3.67 (min:1.46-max:16.92) years. The most common complaints at presentation were headache (68.4%), vision problems (42%), short stature (15%), nausea and vomiting (7%). The surgical procedure applied to the patients was gross total resection (GTR) in 97 cases (63.8%) and subtotal resection in 55 cases (36.2%). Radiotherapy was initiated in 11.8% of the patients. In the pathological examination, 92% of the cases were adamantinamatous type, 8% were papillary type. Postoperatively, hormone deficiencies consisted of thyroid-stimulating hormone (92.1%), adrenocorticotropic hormone (81%), antidiuretic hormone (79%), growth hormone (65.1%), and gonadotropin (43.4%) deficiencies. Recombinant growth hormone treatment (rhGH) was initiated on 27 patients. The study showed hesitancy among physicians regarding rhGH. The median survival without relapse was 2.2 years. Median time of relapse was 1.82 years (range: 0.13-10.35 years). Relapse was related to longer follow-ups and reduced GTR rates. The median follow-up time was 3.13 years. Among the last follow-up visits, the prevalence of obesity was 38%, but of these, 46.5% were already obese at diagnosis. However, 20% who were not obese at baseline became obese on follow-up. Permanent visual impairment was observed in 26 patients, neurological deficits in 13 patients, and diabetes mellitus in 5 patients. Conclusion: Recurrence was predominantly due to incomplete resection and the low rate of postoperative radiotherapy. It also emphasized challenges in multidisciplinary regular follow ups and suggested early interventions such as dietary restrictions and increased exercise to prevent obesity.

9.
J Clin Res Pediatr Endocrinol ; 15(2): 210-213, 2023 05 29.
Artículo en Inglés | MEDLINE | ID: mdl-34565130

RESUMEN

Hereditary angioedema (HAE) is characterized by recurrent angioedema attacks with no urticaria. This disease has a high mortality due to asphyxia. Level of complement component 4 (C4), C1 esterase inhibitor (C1-INH) level and function, and genetic mutations determine different endotypes of HAE. Clinical presentation and the triggers of vasogenic edema may change according to the endotypes. An adolescent girl with oligomenorrhea, obesity, hirsutism, and acanthosis nigricans was diagnosed with polycystic ovary syndrome and prescribed ethinyl estradiol and cyproterone acetate containing oral contraceptive (OC). On the sixteenth day of treatment, she developed angioedema of the face, neck, and chest leading to dyspnea. Adrenaline, antihistamine, and corticosteroid treatments were ineffective. In the family history, the patient's mother and two cousins had a history of angioedema. C1-INH concentrate was administered with a diagnosis of HAE. C4 and C1-INH level and activity were normal. Genetic analysis identified a mutation in the factor 12 (F12) gene, and the diagnosis of F12-related HAE was made. OC treatment was discontinued. She has had no additional angioedema attacks in the follow-up period of two years. OC containing estrogen may induce the life-threatening first attack of F12-related HAE even in children. Recurring angioedema attacks in the family should be asked before prescribing estrogen-containing OC pills.

10.
Diabetes Technol Ther ; 25(10): 689-696, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37449922

RESUMEN

Introduction: The Glycemia Risk Index (GRI) and Continuous Glucose Monitoring Index (COGI) are newly defined composite metric parameters derived from continuous glucose monitoring (CGM) data. GRI is divided into five separate risk zones (from lowest to highest: A-E). In this study, the effect of the advanced hybrid closed loop (AHCL) system on GRI and COGI in children with type 1 diabetes was evaluated. Materials and Methods: Forty-five children who had started using the AHCL and whose baseline and sixth-month CGM data were available were analyzed in terms of achievement of CGM consensus goals and changes in GRI scores and zones. The paired t-test was used for the analyses. Results: The mean age and duration of diabetes of the participants were 10.95 ± 3.41 and 3.85 ± 2.67 years, respectively. The mean GRI score significantly decreased from 35.66 ± 17.46 at baseline to 22.83 ± 9.08 at 6 months (P < 0.001). Although the proportion of those in the A zone was 20% at baseline, it increased to 42% at 6 months. AHCL also improved COGI from 72.59 ± 12.44 to 82.90 ± 7.72 (P < 0.001). Time in range (TIR) increased significantly from 70.54% to 80.51% (P < 0.001) at 6 months. Conclusion: AHCL provides not only an improvement in TIR but also a significant improvement in both GRI and COGI at 6 months. The incorporation of GRI and COGI alongside TIR may enhance the assessment of the glycemic profile by providing a more comprehensive and in-depth analysis.

11.
J Pediatr Endocrinol Metab ; 36(10): 949-956, 2023 Oct 26.
Artículo en Inglés | MEDLINE | ID: mdl-37623928

RESUMEN

OBJECTIVES: This cross-sectional study examined the relationship between problem-solving skills, glucose regulation, and disease management in children with type 1 diabetes mellitus (T1DM) as well as the role of depression in this association. METHODS: The participants (n=54) were recruited from a diabetes camp. Problem-solving inventory (PSI), Beck depression inventory (BDI), and diabetes self-management profile (DSMP) were administered as tests. Forty-six participants who have been diagnosed with T1DM for at least a year and completed the survey were included in the analyses. Participants were categorized into high and low depression groups based on the median split. Student's t-test was used to detect demographic differences in groups. Linear regression models were used to examine the association between PSI, HbA1c, and DSMP. Regressions for PSI and DSMP were repeated in low and high-depression groups. RESULTS: Of the 46 participants with T1DM, 52 % were female, with a mean age of 13.96 ± 1.94 (range 10-17). Avoidant and monitoring style of problem solving as well as the total score of PSI significantly predicted HbA1c levels. Impulsive and avoidant style of problem solving, problem-solving confidence, and total scores of PSI significantly predicted DSMP (p<0.05). The high depression group had a significantly higher DSMP score than the low depression group (p=0.001), with no difference in HbA1c levels (p=0.968). When the DSMP regressions were repeated, no significant associations were seen in the low depression group. Avoidant style of problem solving, problem-solving confidence, and the total score of PSI significantly predicted DSMP in the high depression group (p<0.005). CONCLUSIONS: Problem solving-skills are essential in children with T1DM for a successful disease management. Depression modulates the association between the problem-solving and self-management profile.

12.
J Diabetes Sci Technol ; : 19322968221106194, 2022 Jun 30.
Artículo en Inglés | MEDLINE | ID: mdl-35771030

RESUMEN

BACKGROUND: The insulin requirements of people with type 1 diabetes (T1D) can vary throughout the day due to factors such as biorhythm, exercise, and food intake. The MiniMed 780G system delivers micro boluses to adjust basal insulin and delivers auto-correction boluses to meet insulin needs when micro bolus increases are insufficient. Through analysis of MiniMed 780G data, this study investigates the variations in insulin requirements throughout the day. METHODS: 4193 days' pump and continuous glucose monitoring (CGM) data of 34 children using MiniMed 780G were collected from Medtronic CareLink. Micro and auto-correction boluses were analyzed on an hourly basis for two age groups: below nine years old and above nine years old. Glycemic metrics were analyzed based on International CGM consensus. RESULTS: The mean age was 12.3 years and mean duration of diabetes was 6.1 years. The mean time in range (TIR) and glucose management indicator (GMI) were 80.5% and 6.6%, respectively. The micro bolus (basal) ratio between 05.00 and 07.00 was significantly higher than the ratio between 10.00 and 03.00 (P < .01), whereas micro bolus was significantly lower between 19.00 and 21.00 than those between 00.00 and 10.00 (P < .001). The auto-correction ratio between 21.00 and 00.00 was significantly higher than those between 03.00-17.00 (P < .001) and 19.00-21.00 (P = .008), whereas auto-correction was significantly lower between 07.00 and 10.00 than those between 10.00 and 03.00 (P < .001). The micro bolus ratio was significantly higher in children below nine years old than in children above nine years old between 21.00-00.00 (P = .026) and 00.00-03.00 (P = .003). CONCLUSION: The basal insulin need follows a diurnal pattern with two significantly different periods-high between 00.00 and 10.00 and low between 10.00 and 00.00. The auto-correction rates are low between 05.00 and 10.00 and show an increasing pattern peaking between 21.00 and 00.00. These findings are compatible with the dawn and reverse dawn phenomena.

13.
Ital J Pediatr ; 48(1): 144, 2022 Aug 13.
Artículo en Inglés | MEDLINE | ID: mdl-35964090

RESUMEN

BACKGROUNDS: During the Coronavirus-19 disease (Covid-19) pandemic it was observed that the number of girls presenting with early puberty had increased. The aim of this study was to carry out a retrospective evaluation of the characteristics of girls who had been referred for evaluation of precocious puberty in five different pediatric endocrinology units, before and during the pandemic. METHODS: The study participants comprised 359 girls who were assigned into 2 groups a pre-pandemic group (n:214) and a pandemic group (n:145). Those participants (n:99) who had medical records in the follow-up period were classified into 3 subgroups according to the time of presentation and follow-up visits (group-1: first admission and follow-up visit before the pandemic, group-2: first admission before the pandemic, the follow-up visit during the pandemic, group-3: first admission and follow-up visit during the pandemic). RESULTS: The age at presentation and age at pubertal onset were both significantly lower in the pandemic group than those in the pre-pandemic group(8.1 vs 8.6, p: < 0.001,7.7 vs 7.9,p:0.013, respectively). There was no significant difference between the body mass index standard deviation scores (BMI-SDS) values of the groups (0.57 vs 0.51, p:0.430). The initiation rate of pubertal suppression therapy at the time of presentation was significantly higher in the pandemic group compared to that of the pre-pandemic group (7.7%vs 27.5%), and in groups-2 & 3 compared to group-1, during follow-up (20%&44%vs 8%). CONCLUSION: Our research showed that the onset of puberty occurred earlier in the pandemic period compared to the previous year, and the need for pubertal suppression therapy increased during the pandemic.


Asunto(s)
COVID-19 , Pubertad Precoz , COVID-19/epidemiología , Niño , Estudios Transversales , Femenino , Humanos , Pandemias , Pubertad , Pubertad Precoz/diagnóstico , Pubertad Precoz/epidemiología , Estudios Retrospectivos , Turquía/epidemiología
14.
J Clin Endocrinol Metab ; 107(1): e106-e117, 2022 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-34415991

RESUMEN

BACKGROUND: Aldosterone synthase deficiency (ASD) caused by mutations in the CYP11B2 gene is characterized by isolated mineralocorticoid deficiency. Data are scarce regarding clinical and biochemical outcomes of the disease in the follow-up. OBJECTIVE: Assessment of the growth and steroid profiles of patients with ASD at the time of diagnosis and after discontinuation of treatment. DESIGN AND METHOD: Children with clinical diagnosis of ASD were included in a multicenter study. Growth and treatment characteristics were recorded. Plasma adrenal steroids were measured using liquid chromatography-mass spectrometry. Genetic diagnosis was confirmed by CYP11B2 gene sequencing and in silico analyses. RESULTS: Sixteen patients from 12 families were included (8 females; median age at presentation: 3.1 months, range: 0.4 to 8.1). The most common symptom was poor weight gain (56.3%). Median age of onset of fludrocortisone treatment was 3.6 months (range: 0.9 to 8.3). Catch-up growth was achieved at median 2 months (range: 0.5 to 14.5) after treatment. Fludrocortisone could be stopped in 5 patients at a median age of 6.0 years (range: 2.2 to 7.6). Plasma steroid profiles revealed reduced aldosterone synthase activity both at diagnosis and after discontinuation of treatment compared to age-matched controls. We identified 6 novel (p.Y195H, c.1200 + 1G > A, p.F130L, p.E198del, c.1122-18G > A, p.I339_E343del) and 4 previously described CYP11B2 variants. The most common variant (40%) was p.T185I. CONCLUSIONS: Fludrocortisone treatment is associated with a rapid catch-up growth and control of electrolyte imbalances in ASD. Decreased mineralocorticoid requirement over time can be explained by the development of physiological adaptation mechanisms rather than improved aldosterone synthase activity. As complete biochemical remission cannot be achieved, a long-term surveillance of these patients is required.


Asunto(s)
Citocromo P-450 CYP11B2/deficiencia , Citocromo P-450 CYP11B2/genética , Fludrocortisona/farmacología , Hipoaldosteronismo/patología , Mutación , Privación de Tratamiento/estadística & datos numéricos , Estudios de Casos y Controles , Femenino , Estudios de Seguimiento , Humanos , Hipoaldosteronismo/tratamiento farmacológico , Hipoaldosteronismo/enzimología , Lactante , Recién Nacido , Masculino , Pronóstico
15.
J Clin Res Pediatr Endocrinol ; 14(1): 1-9, 2022 03 03.
Artículo en Inglés | MEDLINE | ID: mdl-34538045

RESUMEN

It is well-known that in children with type 1 diabetes (T1D), the frequency of Celiac disease (CD) is increased due to mechanisms which are not fully elucidated but include autoimmune injury as well as shared genetic predisposition. Although histopathologic examination is the gold standard for diagnosis, avoiding unnecessary endoscopy is crucial. Therefore, for both clinicians and patients' families, the diagnosis of CD remains challenging. In light of this, a joint working group, the Type 1 Diabetes and Celiac Disease Joint Working Group, was convened, with the aim of reporting institutional data and reviewing current international guidelines, in order to provide a framework for clinicians. Several controversial issues were discussed: For CD screening in children with T1D, regardless of age, it is recommended to measure tissue transglutaminase-immunoglobulin A (tTG-IgA) and/or endomysial-IgA antibody due to their high sensitivity and specificity. However, the decision-making process based on tTG-IgA titer in children with T1D is still debated, since tTG-IgA titers may fluctuate in children with T1D. Moreover, seronegativity may occur spontaneously. The authors' own data showed that most of the cases who have biopsy-proven CD had tTG-IgA levels 7-10 times above the upper limit. The decision for endoscopy based solely on tTG-IgA levels should be avoided, except in cases where tTG-IgA levels are seven times and above the upper limit. A closer collaboration should be built between divisions of pediatric endocrinology and gastroenterology in terms of screening, diagnosis and follow-up of children with T1D and suspicious CD.


Asunto(s)
Enfermedad Celíaca , Diabetes Mellitus Tipo 1 , Autoanticuerpos , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/diagnóstico , Niño , Toma de Decisiones Clínicas , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 1/diagnóstico , Humanos , Inmunoglobulina A , Transglutaminasas
16.
Turk J Pediatr ; 53(4): 464-6, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21980854

RESUMEN

Skeletal dysplasias (osteochondrodysplasias) are a group of diseases that must be included in the differential diagnosis of disproportionate short stature. History, clinical and radiologic findings and consanguinity are important features to be considered when a specific diagnosis is investigated. Spondyloenchondrodysplasia is a very rare skeletal dysplasia characterized with enchondromas in the long bones and platyspondyly. Manifestation of the disorder may include neurological involvement (spasticity, intracranial calcifications and mental retardation) and immune dysfunction. Herein, we report a 12-year-old boy who admitted to our clinic with short stature, who was born to consanguineous parents. He presented clinical (significant widening of wrists, ankles and knees) and radiologic (enchondromatous lesions in the metaphysis of long bones) features of spondyloenchondrodysplasia but did not yet have neurologic or immunologic involvement.


Asunto(s)
Enfermedades Autoinmunes/diagnóstico por imagen , Osteocondrodisplasias/diagnóstico por imagen , Estatura , Niño , Consanguinidad , Diagnóstico Diferencial , Humanos , Masculino , Radiografía
17.
J Clin Res Pediatr Endocrinol ; 13(3): 285-292, 2021 08 23.
Artículo en Inglés | MEDLINE | ID: mdl-33491925

RESUMEN

Objective: Hypoglycemia is a common acute complication of type 1 diabetes (T1D), which may cause seizure, loss of consciousness, and temporary motor or sensory impairment. Glucagon administration is an effective way of treating severe hypoglycemia, especially in a free-living setting. Nonetheless, families have difficulties in managing severe hypoglycemia due to their anxiety and challenges with current glucagon administration techniques. The aim of the current study was to explore the associations between parental fear of hypoglycemia (FoH) and their general anxiety level, and in particular, their attitudes towards and thoughts on glucagon administration. Methods: Parents of children with T1D completed questionnaires assessing background and clinical information, FoH, generalized anxiety disorder (GAD) and parental anxiety for glucagon administration (PAGA). Results: Sixty-eight parents participated. Positive correlations were found between parental GAD-7 score and both FoH and the number of night-time blood glucose measurements and there was a negative correlation with the child's age. Parents mean self-evaluation score of their competence in glucagon administration was 6 (standard deviation±2.9) on a scale of 0 to 10. Unsurprisingly, this score was negatively correlated with the PAGA scores. There was no significant difference between children using continuous glucose monitoring system and self-monitoring of blood glucose in terms of parental FoH, anxiety and misconceptions about glucagon administration. Conclusion: The results showed that parents of children with T1D had anxiety and fear connected with hypoglycemia and glucagon administration. Structured and practical training should be implemented to increase parents' self-confidence including annual refresher training for home glucagon administration.


Asunto(s)
Ansiedad/etiología , Glucemia/efectos de los fármacos , Cuidadores/psicología , Diabetes Mellitus Tipo 1/tratamiento farmacológico , Miedo , Glucagón/administración & dosificación , Hipoglucemia/tratamiento farmacológico , Hipoglucemiantes/efectos adversos , Padres/psicología , Adolescente , Ansiedad/diagnóstico , Ansiedad/psicología , Biomarcadores/sangre , Glucemia/metabolismo , Niño , Preescolar , Diabetes Mellitus Tipo 1/sangre , Diabetes Mellitus Tipo 1/diagnóstico , Femenino , Glucagón/efectos adversos , Conocimientos, Actitudes y Práctica en Salud , Humanos , Hipoglucemia/sangre , Hipoglucemia/inducido químicamente , Hipoglucemia/diagnóstico , Inyecciones Intramusculares , Masculino , Proyectos Piloto , Encuestas y Cuestionarios
18.
J Clin Res Pediatr Endocrinol ; 13(4): 370-374, 2021 11 25.
Artículo en Inglés | MEDLINE | ID: mdl-34013712

RESUMEN

Diabetes care at school has recently appeared on the agenda of international diabetes organizations, the basic principles of which have been newly determined. The aim of this review was to summarize the activities and output of the Diabetes at School Program - a program that has been delivered in Turkey for the last 10 years - and to focus on different aspects of Diabetes Care at School through a national model. Recently, a detailed set of national regulations, including the basic principles proposed by the International Society for Pediatric and Adolescent Diabetes and the experience in Turkey, was prepared and has come into force. The future agenda includes giving priority to socio-economically disadvantaged regions, provision of an Individual Treatment Plan at School for each child with diabetes and ensuring that each school has an action plan for the care of children with diabetes. We believe that if all countries have programs and structured national regulations similar to the Diabetes at School Program, this will enable significant progress in the level of care delivered to children with diabetes.


Asunto(s)
Diabetes Mellitus Tipo 1/terapia , Desarrollo de Programa , Instituciones Académicas , Adolescente , Niño , Femenino , Humanos , Masculino , Turquía
19.
J Patient Exp ; 8: 23743735211056523, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34881352

RESUMEN

Investigating the daily life experiences of patients using Continuous Glucose Monitoring (CGM) can highlight the benefits and barriers in using this system for people with type 1 diabetes (T1D). Semi-structured qualitative interviews were conducted with the caregivers of 10 children aged <9 years, all of whom had been treated for T1D and had used CGM >6 months. These interviews were analyzed using the content analysis approach and from these interviews, four meta themes emerged: metabolic control, barriers to CGM use, CGM use in daily life, and comparison with fingersticks. Families reported the following as benefits of CGM: pain relief, better hypoglycemia and hyperglycemia management, increased control over diet and social life, reduced worries at school and during the night, and convenience in entrusting the child to the care of others. Cost, concerns related to accuracy and reliability of measurements, insertion, adhesion and removal issues all emerged as barriers to CGM use. The most prominent issue was the economic burden of CGM. Families accept this burden, even though it is challenging, as their experiences in using CGM are positive and they feel that CGM is necessary for T1D management.

20.
J Clin Res Pediatr Endocrinol ; 12(3): 248-255, 2020 09 02.
Artículo en Inglés | MEDLINE | ID: mdl-31893581

RESUMEN

Objective: The aim was to determine vitamin D status in the general population in Turkey between 2011 and 2016, and to evaluate the effectiveness of the national vitamin D supplementation programme. Methods: Serum 25-hydroxyvitamin D (25-OHD) measurement data were retrieved from an internationally accredited laboratory, operating nationwide. A total of 108,742 measurements of 25-OHD were analyzed using the cut-off values of 0-11 ng/mL, 12-19 ng/mL, 20-49 ng/mL, 50-70 ng/mL and >70 ng/mL for vitamin D deficiency, insufficiency, sufficiency, possibly harmful and excess respectively. Results: The mean±standard deviation 25-OHD level was 21.6±13.3 ng/mL. Mean 25-OHD concentrations by age groups were: 37.3 ng/mL, 30.1 ng/mL and 23.7 ng/mL for <1, 1-10 and 11-18 year old groups, respectively. Mean 25-OHD levels of children <1 year and 1-3 years of age were significantly higher than those found in other age groups. The prevalence of vitamin D deficiency (<12 ng/mL) was lowest in children at 1-3 years of age (5%). In subjects older than 18 years of age, mean 25-OHD levels were 18.2 ng/mL, 20.1 ng/mL, 21.9 ng/mL and 21.1 ng/mL for age groups 19-30, 31-50, 51-70 and >70 years, respectively. Conclusion: Successful implementation of the national vitamin D supplementation programme, appears to have nearly eliminated vitamin D deficiency for children under 1-years of age. However, the positive impact of the vitamin D supplementation diminishes as children get older suggesting that supplementation may be required in the older children and adults. In addition, improved awareness of the benefits and risks of excess vitamin D should prevent unnecessary and excessive use of vitamin D supplements.


Asunto(s)
Deficiencia de Vitamina D/sangre , Deficiencia de Vitamina D/epidemiología , Vitamina D/sangre , Adolescente , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Envejecimiento/sangre , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Laboratorios , Masculino , Persona de Mediana Edad , Turquía/epidemiología , Vitamina D/análogos & derivados , Deficiencia de Vitamina D/diagnóstico , Adulto Joven
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