Importance of Growth Factors and Bone Maturation Ratio in the Response to Growth Hormone Therapy.

OBJECTIVE: The aim was to identify the influence of insulin-like growth factor I (IGF-1), IGF-binding protein-3 (IGFBP-3), and bone age (BA)/chronological age (CA) ratio on the response to GH therapy after 1 and 2 years of treatment and upon reaching final height. METHODS: Longitudinal, retrospective, observational study of 139 patients treated for idiopathic growth hormone deficiency. Variables examined during follow-up: (1) genetic background; (2) perinatal history; (3) anthropometry; (4) height velocity, BA, BA/CA and height prognosis; (5) analytical results (IGF-1, IGFBP-3). Final response variables: adult height (AH), AH with respect to target height, AH with respect to initial height prognosis, AH with respect to height at the start of treatment, and AH with respect to height at onset of puberty. RESULTS: Lower pretreatment IGF-1 levels and a greater increase in IGF-1 at the end of treatment imply a better response (r = -0.405, P = .007 and r = 0.274, P = .014, respectively), as does a greater increase in IGFBP-3 after 2 years of treatment and at the end of treatment (r = 0.207, P = .035 and r = 0.259, P = .020, respectively). A lower BA/CA ratio pretreatment and at the onset of puberty results in a better response (r = -0.502, P = .000 and r = -0.548, P = .000, respectively), as does a lower increase in BA and BA/CA ratio after the 1 and 2 years of treatment (r = -0.337, P = .000 and r = -0.332, P = .000, respectively). CONCLUSION: Low pretreatment IGF-1, a greater BA delay with respect to CA pretreatment and at the onset of puberty, a greater increase in IGFBP-3 after 2 years of treatment, and a lower increase in BA and BA/CA ratio after 1 and 2 years of treatment imply a better long-term response.

Translation and psychometric evaluation of the Spanish version of the problem areas in diabetes-pediatric version (PAID-Peds) survey.

BACKGROUND: Metabolic control and psychological management of paediatric type 1 diabetes mellitus (T1DM) can be challenging over time. Development of an instrument to assess the youth-reported burden could aid in preventing T1DM-associated diseases. METHODS: The aim of this study was to translate and validate the Spanish version of the Problem Area in Diabetes Survey-Pediatric version (PAID-Peds). A multicentre, cross-sectional translation and linguistic validation study was performed on a sample of 30 participants aged 8-17 years with a minimum 1-year history of T1DM diagnosed at the Miguel Servet University Hospital in Zaragoza (Aragon, Spain), Ramón y Cajal University Clinical Hospital in Madrid (Spain), and Sant Joan de Déu Hospital in Barcelona (Catalonia, Spain). The qualitative validation consisted of translation into Spanish and back-translation into English of the Paid-Peds survey and subsequent administration to the sample population. Data were gathered on parameters related to sociodemographic characteristics and metabolic control. Validity, feasibility, and test-retest reliability were evaluated. Internal consistency was determined using Cronbach's alpha coefficient, test-retest reliability by means of interclass correlation, and paired samples using the Wilcoxon W-test. The study was approved by the ethics and research committees at each participating centre. RESULTS: The study assessed 30 children (46.7% female) with an average age of 13.33 ± 2.98 years; mean age at onset was 5.70 ± 3.62 years, and the mean disease duration was 7.63 ± 4.36 years. The mean score on the PAID-Peds survey was 42.88 ± 17.85. Cronbach's alpha coefficient was 0.90. Test-retest reliability measured by interclass correlation coefficient was 0.8 (95% CI: 0.63-0.90). No significant differences in total scores were found between test and retest (Wilcoxon W-test: 289; p = 0.051). CONCLUSIONS: The Spanish version of the PAID-Peds survey is a feasible, valid, and reliable instrument to assess the youth-perceived burden of T1DM.

Increased Presentation of Diabetic Ketoacidosis and Changes in Age and Month of Type 1 Diabetes at Onset during the COVID-19 Pandemic in Spain.

Objective: To assess the impact of the COVID-19 pandemic and lockdown measures on the presenting characteristics (age at diagnosis, severity, monthly distribution) of newly diagnosed type 1 diabetes in Spanish children. Research Design and Methods: An ambispective observational multicenter study was conducted in nine Spanish tertiary-level hospitals between January 2015 and March 2021. Inclusion criteria: new cases of type 1 diabetes in children (0-14 years) recording age, sex, date of diagnosis, presence of diabetic ketoacidosis (DKA) at onset, and severity of DKA. Data were compared before and during the pandemic. Results: We registered 1444 new cases of type 1 diabetes in children: 1085 in the pre-pandemic period (2015-2019) and 359 during the pandemic (2020-March 2021). There was a significant increase in the group aged ≤4 years in the pandemic period (chi-squared = 10.986, df 2, p = 0.0041). In 2020-2021, cases of DKA increased significantly by 12% (95% CI: 7.2-20.4%), with a higher percentage of moderate and severe DKA, although this increase was not significant. In 2020, there was a sharp decrease in the number of cases in March, with a progressive increase from May through November, higher than in the same months of the period 2015-2019, highlighting the increase in the number of cases in June, September, and November. The first three months of 2021 showed a different trend to that observed both in the years 2015-2019 and in 2020, with a marked increase in the number of cases. Conclusions: A change in monthly distribution was described, with an increase in DKA at onset of type 1 diabetes. No differences were found in severity, although there were differences in the age distribution, with an increase in the number of cases in children under 4 years of age.

Intralymphatic GAD-Alum (Diamyd®) Improves Glycemic Control in Type 1 Diabetes With HLA DR3-DQ2.

AIMS: Residual beta cell function in type 1 diabetes (T1D) is associated with lower risk of complications. Autoantigen therapy with GAD-alum (Diamyd) given in 3 intralymphatic injections with oral vitamin D has shown promising results in persons with T1D carrying the human leukocyte antigen (HLA) DR3-DQ2 haplotype in the phase 2b trial DIAGNODE-2. We aimed to explore the efficacy of intralymphatic GAD-alum on blood glucose recorded by continuous glucose monitoring (CGM). METHODS: DIAGNODE-2 (NCT03345004) was a multicenter, randomized, placebo-controlled, double-blind trial of 109 recent-onset T1D patients aged 12 to 24 years with GAD65 antibodies and fasting C-peptide > 0.12 nmol/L, which randomized patients to 3 intralymphatic injections of 4 µg GAD-alum and oral vitamin D, or placebo. We report results for exploratory endpoints assessed by 14-day CGM at months 0, 6, and 15. Treatment arms were compared by mixed-effects models for repeated measures adjusting for baseline values. RESULTS: We included 98 patients with CGM recordings of sufficient quality (DR3-DQ2-positive patients: 27 GAD-alum-treated and 15 placebo-treated). In DR3-DQ2-positive patients, percent of time in range (TIR, 3.9-10 mmol/L) declined less between baseline and month 15 in GAD-alum-treated compared with placebo-treated patients (-5.1% and -16.7%, respectively; P = 0.0075), with reduced time > 13.9 mmol/L (P = 0.0036), and significant benefits on the glucose management indicator (P = 0.0025). No differences were detected for hypoglycemia. GAD-alum compared to placebo lowered the increase in glycemic variability (standard deviation) observed in both groups (P = 0.0219). Change in C-peptide was correlated with the change in TIR. CONCLUSIONS: Intralymphatic GAD-alum improves glycemic control in recently diagnosed T1D patients carrying HLA DR3-DQ2.

Influence of the SARS-CoV-2 pandemic on paediatric patients with type 1 diabetes mellitus after one year of follow-up.

OBJECTIVES: Lockdown during the SARS-CoV-2 pandemic generated uncertainty regarding its effects on the control of type 1 diabetes (DM1). Our study aims to evaluate the influence of the pandemic on the control of paediatric patients with DM1. METHODS: Longitudinal, retrospective, observational study in patients with DM1 attended between 15/10/2019 and 15/03/2020. Data were collected at that visit and at the three subsequent visits. The second was remote in 50% of cases. The variables analysed were: type of insulin therapy, time in range (TIR), time in hypoglycaemia (THypo), time in hyperglycaemia (THyper), coefficient of variation (CV), glycosylated haemoglobin, insulin requirements and anthropometric data. RESULTS: 157 patients were recruited. At the post-lockdown visit, the TIR increased and the THyper decreased with respect to the first (p<0.00) and second (p<0.00) visits. Patients treated with subcutaneous infusion showed a higher TIR at the third visit (p=0.03) and lower insulin requirements at the fourth visit (p=0.03) compared to patients treated with multiple doses. Patients with a remote visit presented a higher TIR (p<0.00), a lower THyper (p=0.00) and lower insulin requirements (p=0.01) at the next visit. Patients aged less than 6 years presented a lower glycosylated haemoglobin (p=0.01) and insulin requirements at the third (p=0.03) and fourth (p=0.01) visits, and a lower increase in body mass index (p=0.03) over the year. CONCLUSIONS: Metabolic control improved at the post-lockdown visit. Patients treated with subcutaneous infusion, those who had a remote visit during strict lockdown and those aged less than 6 years had a better evolution.

Growth hormone deficit: Influence of puberty on the response to treatment.

INTRODUCTION: Short stature is the most frequent reason for consultation in Pediatric Endocrinology consultations and sometimes requires treatment with growth hormone. The aim of the study was to analyze the response to treatment based on its onset in pubertal or prepubertal stages and to analyze the possible benefit of an early onset. PATIENTS AND METHODS: Longitudinal, retrospective and observational study in 139 patients treated for idiopathic growth hormone deficiency up to adult height. MAIN VARIABLES STUDIED: (a) genetic background: maternal, paternal and genetic height; (b) perinatal history; (c) anthropometry during follow-up and at pubertal onset: weight, height, body mass index; (d) variables during follow-up and at pubertal onset: growth rate, bone age and growth prognosis. Final response variables: adult height, adult height with respect to target height, adult height with respect to initial growth prediction, adult height with respect to initial height at the start of treatment and adult height with respect to height at pubertal onset. RESULTS: Total pubertal gain was 0.84 ± 0.6 SD. 61.9% of the patients started treatment with rhGH in prepuberty. The initiation of treatment in the prepubertal stage and a higher total pubertal gain are correlated with a better final height (P = 0.001 and r = 0.507, P = 0.00, respectively). Furthermore, a longer duration of treatment in pre-puberty is correlated with a better final response (r = 0.328, P = 0.00). CONCLUSIONS: The start of treatment in the prepubertal stage and its longer duration during this period are determining factors to achieve a good long-term response. Total pubertal gain was greater in patients who started treatment in the pubertal stage.

Growth hormone deficit. Does the first year of treatment influence adult height?

INTRODUCTION: Short stature is the most frequent reason for Pediatric Endocrinology consultations and sometimes requires treatment with growth hormone. OBJECTIVE: The possible correlation of a good response to any early response factor with a better final response was studied, and also whether there was a difference in response to treatment according to the type of deficit. PATIENTS AND METHODS: This was a longitudinal, retrospective and observational study of 139 patients treated for idiopathic growth hormone deficiency up to adult height. There were good response criteria in the first year of treatment: a) an increase in growth rate ≥3 cm/year, b) a growth rate ≥1 standard deviation (SD), c) an increase in height ≥0.5 SD, d) an increase in height ≥0.3 SD. Study of the Index of Responsiveness to treatment in the first and second year. Final response variables: adult height with respect to target height, adult height with respect to initial growth prediction and adult height with respect to initial height at the start of treatment. The possible correlation of a good response to any of the early response factors with a better final response to treatment was studied, and also whether there was a difference in the response to treatment according to the type of deficit. RESULTS: The treatment produced a gain in adult height with respect to genetic height of 0.06 ±â€¯0.7 SD. Patients considered good responders in the first year of treatment presented a better final response (growth rate ≥3 cm: p = 0.000, growth rate ≥1 SD: p = 0.008, height gain ≥0.5 SD: p = 0.007, height gain ≥0.3 SD: p = 0.006), as well as patients with a severe deficit (p = 0.04). The index of responsiveness to treatment during the first year was associated with a better final response (r = 0.249, p = 0.003), with this correlation being maintained in the second year (r = 0.294, p = 0.01). CONCLUSIONS: Growth hormone treatment increased height in the genetic target. The percentage of good responders varied depending on the criteria used. The response in the first year of treatment and a severe deficit were determining factors for achieving a good long-term response.

The impact of "faster aspart" on blood glucose control in children and adolescents with type 1 diabetes treated using a sensor-augmented insulin pump.

BACKGROUND AND AIMS: Post-prandial glucose control is essential to achieve metabolic goals in patients with type 1 diabetes mellitus (T1DM). The new "faster aspart" insulin has a pharmacological profile noted for its faster absorption and onset of action, and increased early availability, resulting in improved blood glucose control after meals. The main objective of the study was to analyse the efficacy of "faster aspart" vs. "insulin aspart" in children and adolescents with DM1 on sensor-augmented pump treatment. PATIENTS AND METHODS: Multicentre, longitudinal and prospective analytical trial evaluating the use of faster aspart insulin for three months in children with T1DM with MiniMed640G® sensor-augmented pumps previously treated with aspart insulin. At the beginning and end of the study the following variables were analysed for subsequent comparison: mean sensor glucose, percentage of time in range, hypoglycaemia and hyperglycaemia, area under the curve (AUC) <70 and >180 mg/dL, mean sensor glucose pre- and postprandial in main meals, daily insulin requirements, basal/bolus percentage, and HbA1c. Acute complications, adverse events and satisfaction survey were assessed. RESULTS: The study included 32 patients with a mean of 13.49 ± 2.42 years of age and with T1DM of 7.0 ± 3.67 years of onset. The use of faster aspart was associated with lower time in hyperglycaemia >180 mg/dL (25.8 ± 11.3 vs. 22.4 ± 9.5; p = .011) and >250 mg/dL (5.2±4.9 vs. 4.0 ± 3.6; p = .04), lower AUC >180 mg/dL (10.8 ± 6.5 vs. 9.3 ± 6.1; p = .03), and increased time in range (71.4 ± 10.0 vs. 74.3 ± 9.2; p = .03). No significant changes in hypoglycaemia, HbA1c, insulin requirements, and basal/bolus percentages were detected. Faster aspart was safe and well-evaluated by patients and caregivers. CONCLUSIONS: Faster aspart achieves better glycaemic control by increasing glucose time in range in children and adolescents with T1DM on treatment with sensor-augmented pumps.

A Highly Efficient Neutral Anion Receptor in Polar Environments by Synergy of Anion-π Interactions and Hydrogen Bonding.

Herein, it is shown how anion recognition in highly polar solvents by neutral metal-free receptors is feasible when multiple hydrogen bonding and anion-π interactions are suitably combined. A neutral aromatic molecular tweezer functionalized with azo groups is shown to merge these two kinds of interactions in a unique system and its efficiency as an anion catcher in water is evaluated using first-principles quantum methods. Theoretical calculations unequivocally prove the high thermodynamic stability in water of a model anion, bromide, captured within the tweezer's cavity. Thus, static calculations indicate anion-tweezer interaction energies within the range of covalent or ionic bonds and stability constants in water of more than 10 orders of magnitude. First-principles molecular dynamics calculations also corroborate the stability through the time of the anion-tweezer complex in water. It shows that the anion is always found within the tweezer's cavity due to the combination of the tweezer-anion interactions plus a hydrogen bond between the anion and a water molecule that is inside the tweezer's cavity.

Impact on the well-being perceived by caregivers of children and adolescents with type 1 diabetes following the use of interstitial glucose measurement systems.

INTRODUCTION: Type 1 diabetes mellitus (DM-1) is one of the most common chronic childhood diseases, and it is essential to optimize glycemic control in order to avoid complications. For years, interstitial glucose measurement systems (MGI systems) have been among the new technologies at the forefront of self-care. OBJECTIVES: To determine the impact on the well-being of the caregivers of patients with DM-1 under 18 years of age, controlled at a Pediatric Diabetes Unit of a third level hospital, of the use of MGI systems. MATERIAL AND METHODS: This was an observational, descriptive and analytical cohort study based on a questionnaire completed by the patients' caregivers, as well as from the patient's clinical history. RESULTS: There were 120 participants (55.5% males), with a mean age 13.20 +/- 3.71 years and mean glycosylated haemoglobin (HbA1c) 7.36% +/- 0.90. 52.5% of the sample used MGI systems. The caregivers of patients using MGI systems showed significantly higher scores (p < 0.05) regarding well-being, compared to the caregivers of patients not using this technology. In the former, a significant improvement (p < 0.05) in these variables with respect to the values prior to the beginning of their use was observed. CONCLUSIONS: The use of MGI systems for diabetes self-management in our study led to a greater sense of well-being on the part of caregivers compared with before their introduction, as well as in comparison with those who continued to perform measurements using daily capillary glycemias.

Intralymphatic Glutamic Acid Decarboxylase With Vitamin D Supplementation in Recent-Onset Type 1 Diabetes: A Double-Blind, Randomized, Placebo-Controlled Phase IIb Trial.

OBJECTIVE: To evaluate the efficacy of aluminum-formulated intralymphatic glutamic acid decarboxylase (GAD-alum) therapy combined with vitamin D supplementation in preserving endogenous insulin secretion in all patients with type 1 diabetes (T1D) or in a genetically prespecified subgroup. RESEARCH DESIGN AND METHODS: In a multicenter, randomized, placebo-controlled, double-blind trial, 109 patients aged 12-24 years (mean ± SD 16.4 ± 4.1) with a diabetes duration of 7-193 days (88.8 ± 51.4), elevated serum GAD65 autoantibodies, and a fasting serum C-peptide >0.12 nmol/L were recruited. Participants were randomized to receive either three intralymphatic injections (1 month apart) with 4 µg GAD-alum and oral vitamin D (2,000 IE daily for 120 days) or placebo. The primary outcome was the change in stimulated serum C-peptide (mean area under the curve [AUC] after a mixed-meal tolerance test) between baseline and 15 months. RESULTS: Primary end point was not met in the full analysis set (treatment effect ratio 1.091 [CI 0.845-1.408]; P = 0.5009). However, GAD-alum-treated patients carrying HLA DR3-DQ2 (n = 29; defined as DRB1*03, DQB1*02:01) showed greater preservation of C-peptide AUC (treatment effect ratio 1.557 [CI 1.126-2.153]; P = 0.0078) after 15 months compared with individuals receiving placebo with the same genotype (n = 17). Several secondary end points showed supporting trends, and a positive effect was seen in partial remission (insulin dose-adjusted HbA1c ≤9; P = 0.0310). Minor transient injection site reactions were reported. CONCLUSION: Intralymphatic administration of GAD-alum is a simple, well-tolerated treatment that together with vitamin D supplementation seems to preserve C-peptide in patients with recent-onset T1D carrying HLA DR3-DQ2. This constitutes a disease-modifying treatment for T1D with a precision medicine approach.

[Growth hormone deficit: Influence of puberty on the response to treatment]. / Déficit de hormona de crecimiento: influencia de la pubertad en la respuesta al tratamiento.

INTRODUCTION: Short stature is the most frequent reason for consultation in Pediatric Endocrinology consultations and sometimes requires treatment with growth hormone. The aim of the study was to analyze the response to treatment based on its onset in pubertal or prepubertal stages and to analyze the possible benefit of an early onset. PATIENTS AND METHODS: Longitudinal, retrospective and observational study in 139 patients treated for idiopathic growth hormone deficiency up to adult height. MAIN VARIABLES STUDIED: (a) genetic background: maternal, paternal and genetic height; (b) perinatal history; (c) anthropometry during follow-up and at pubertal onset: weight, height, body mass index; (d) variables during follow-up and at pubertal onset: growth rate, bone age and growth prognosis. Final response variables: adult height, adult height with respect to target height, adult height with respect to initial growth prediction, adult height with respect to initial height at the start of treatment and adult height with respect to height at pubertal onset. RESULTS: Total pubertal gain was 0.84±0.6 SD. 61.9% of the patients started treatment with rhGH in prepuberty. The initiation of treatment in the prepubertal stage and a higher total pubertal gain are correlated with a better final height (P=.001 and r=0.507, P=.00, respectively). Furthermore, a longer duration of treatment in pre-puberty is correlated with a better final response (r=0.328, P=.00). CONCLUSIONS: The start of treatment in the prepubertal stage and its longer duration during this period are determining factors to achieve a good long-term response. Total pubertal gain was greater in patients who started treatment in the pubertal stage.

Growth hormone deficit. Does the first year of treatment influence adult height? / Déficit de hormona de crecimiento, ¿influye el primer año de tratamiento en la talla final?

INTRODUCTION: Short stature is the most frequent reason for Pediatric Endocrinology consultations and sometimes requires treatment with growth hormone. OBJECTIVE: The possible correlation of a good response to any early response factor with a better final response was studied, and also whether there was a difference in response to treatment according to the type of deficit. PATIENTS AND METHODS: This was a longitudinal, retrospective and observational study of 139 patients treated for idiopathic growth hormone deficiency up to adult height. There were good response criteria in the first year of treatment: a) an increase in growth rate≥3cm / year, b) a growth rate≥1 standard deviation (SD), c) an increase in height≥0.5 SD, d) an increase in height≥0.3 SD. Study of the Index of Responsiveness to treatment in the first and second year. Final response variables: adult height with respect to target height, adult height with respect to initial growth prediction and adult height with respect to initial height at the start of treatment. The possible correlation of a good response to any of the early response factors with a better final response to treatment was studied, and also whether there was a difference in the response to treatment according to the type of deficit. RESULTS: The treatment produced a gain in adult height with respect to genetic height of 0.06±0.7 SD. Patients considered good responders in the first year of treatment presented a better final response (growth rate≥3cm: p=0.000, growth rate≥1 SD: p=0.008, height gain≥0.5 SD: P=0.007, height gain≥0.3 SD: P=0.006), as well as patients with a severe deficit (P=0.04). The index of responsiveness to treatment during the first year was associated with a better final response (r=0.249, P=0.003), with this correlation being maintained in the second year (r=0.294, P=0.01). CONCLUSIONS: Growth hormone treatment increased height in the genetic target. The percentage of good responders varied depending on the criteria used. The response in the first year of treatment and a severe deficit were determining factors for achieving a good long-term response.

Eosinophilic granuloma of cranial location: report of a case and bibliographic review / [Granuloma eosinófilo de localización craneal: reporte de un caso y revisión bibliográfica]

Introduction: Eosinophilic granuloma is an unusual benign disease that usually affects the pediatric population and young adults. It is the most benign of the diseases traditionally known as histiocytosis X that are now called Langerhans cell histiocytosis. Clinical Case: Pediatric patient with a painful lump in the temporal region. The imaging tests carried out reveal the existence of an osteolytic lesion with an aggressive pattern compatible with eosinophilic granuloma. The patient underwent surgery with a conclusive definitive histological diagnosis of eosinophilic granuloma. Discussion: Eosinophilic granuloma can affect one or multiple bones, of which the most frequent are the cranial bones, epiphyses of long bones and ribs, requiring individualized diagnosis and treatment strategies for optimal management and results, with surgical treatment of First choice. Conclusion: Eosinophilic granuloma is an infrequent benign condition that requires a correct anamnesis and clinical examination of the patient, as well as the demonstration of the characteristic radiological images, allowing a generally accurate presumptive diagnosis to be reached that in most cases can be considered definitive.

Introducción: El granuloma eosinófilo es una enfermedad benigna poco usual que suele afectar a la población pediátrica y adultos jóvenes. Es la afección más benigna de las enfermedades tradicionalmente conocidas como histiocitosis X que en la actualidad reciben la denominación de histiocitosis de células de Langerhans. Caso Clínico: Paciente pediátrico con bultoma doloroso en región temporal. Las pruebas de imagen realizadas ponen de manifiesto la existencia de una lesión osteolítica con patrón de agresividad compatible con granuloma eosinófilo. El paciente es intervenido mediante cirugía con diagnóstico histológico definitivo concluyente de granuloma eosinófilo. Discusión: El granuloma eosinófilo puede afectar a uno o múltiples huesos, de los cuáles los más frecuentes son los huesos craneales, epífisis de huesos largos y costillas, precisando estrategias de diagnóstico y tratamiento individualizadas para un manejo y resultado óptimo, siendo el tratamiento quirúrgico de primera elección. Conclusión: El granuloma eosinófilo es un cuadro benigno infrecuente que requiere de una correcta anamnesis y exploración clínica del paciente, así como la demostración de las imágenes radiológicas características, permitiendo llegar a un diagnóstico de presunción generalmente certero que en la mayoría de ocasiones se puede considerar definitivo.

Impact on the well-being perceived by caregivers of children and adolescents with type 1 diabetes following the use of interstitial glucose measurement systems. / Impacto en el bienestar percibido por cuidadores de niños y adolescentes con diabetes tipo 1 mediante la utilización de sistemas de medición de glucosa intersticial.

INTRODUCTION: Type 1 diabetes mellitus (DM-1) is one of the most common chronic childhood diseases, and it is essential to optimize glycemic control in order to avoid complications. For years, interstitial glucose measurement systems (MGI systems) have been among the new technologies at the forefront of self-care. OBJECTIVES: To determine the impact on the well-being of the caregivers of patients with type 1 diabetes mellitus under 18 years of age, controlled at a Pediatric Diabetes Unit of a third level hospital, of the use of MGI systems. MATERIAL AND METHODS: This was an observational, descriptive and analytical cohort study based on a questionnaire completed by the patients' caregivers, as well as from the patient's clinical history. RESULTS: There were 120 participants (55.5% males), with a mean age 13.20+/-3.71 years and mean glycosylated haemoglobin (HbA1c) 7.36%+/-0.90. 52.5% of the sample used MGI systems. The caregivers of patients using MGI systems showed significantly higher scores (p<.05) regarding well-being, compared to the caregivers of patients not using this technology. In the former, a significant improvement (p<.05) in these variables with respect to the values prior to the beginning of their use was observed. CONCLUSIONS: The use of MGI systems for diabetes self-management in our study led to a greater sense of well-being on the part of caregivers compared with before their introduction, as well as in comparison with those who continued to perform measurements using daily capillary glycemias.

Osteogenesis imperfecta: Review of 40 patients. / Osteogénesis imperfecta: análisis de 40 pacientes.

INTRODUCTION: Osteogenesis imperfecta (OI) is a heterogeneous genetic disease manifesting as bone fragility and fractures. PATIENTS AND METHODS: Retrospective descriptive study analysing clinical and genetic features, and treatment of patients with OI. RESULTS: Forty patients were included; 32.5% males, 67.5% females; 29 children, 11 adults. Number of fractures at diagnosis with mild OI was 4.6±6.4 (average age at diagnosis 7.8±12.8years), with moderate OI 1.7±2.4 (age at diagnosis .04±.3years), in severe OI 3.7±2.1 and in extremely severe forms 12.5±7.8, both groups diagnosed at birth. Genetic study in 32 patients, 25 with a positive genetic study (pathogenic/probably pathogenic variant). COL1A1 gene was the most frequently affected. In 7 patients, no pathogenic or probably pathogenic variant was found (5 diagnosed by biochemical study of typeI collagen). Nineteen patients were treated with bisphosphonates; 7 combined with growth hormone. The patients treated with bisphosphonates showed clinical improvement (reduction of bone pain and/or irritability) and reduction of fractures. CONCLUSIONS: The COL1A1 gene is the most frequently affected. OI patients should receive multidisciplinary management and bisphosphonates can improve their quality of life.