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1.
J Pediatr Endocrinol Metab ; 33(7): 951-955, 2020 Jul 28.
Artículo en Inglés | MEDLINE | ID: mdl-32447330

RESUMEN

Objectives Prader-Willi Syndrome (PWS) is characterised by hyperphagia often leading to obesity; a known risk factor for insulin resistance and type 2 (T2) diabetes. We present a prepubertal girl with PWS who developed diabetes. Case presentation Our case was diagnosed with PWS in infancy following investigation for profound central hypotonia and feeding difficulties. She commenced growth hormone (GH) aged 8 years for short stature and treatment improved linear growth. At age 12 years, she presented with polydipsia, polyuria and vulvovaginitis. She was overweight (BMI SDS +1.43). Diabetes was diagnosed (Blood glucose = 24.2 mmol/L, HbA1c = 121 mmol/mol or 13.2%). She was not acidotic and had negative blood ketones. Autoantibodies typical of type 1 diabetes were negative. She was initially treated with basal bolus insulin regime. GH was discontinued 3 months later due to concerns regarding GH-induced insulin resistance. Off GH, insulin requirements reduced to zero, allowing Metformin monotherapy. However off GH, she reported significant lethargy with static growth and increased weight. Combinations of Metformin with differing insulin regimes did not improve glucose levels. Liraglutide (GLP-1 agonist) and Metformin did not improve glucose levels nor her weight. Liraglutide and Empaglifozin (SGLT-2 inhibitor) therapy used in combination were well tolerated and demonstrated rapid normalisation of blood glucose and improvement in her HbA1c to within target (48 mmol/mol) which was sustained after 6 months of treatment. Conclusions Newer treatments for type 2 diabetes (e. g. GLP-1 agonists or SGLT-2 inhibitors) offer potential treatment options for those with diabetes and PWS when conventional treatments are ineffective.


Asunto(s)
Compuestos de Bencidrilo/administración & dosificación , Diabetes Mellitus/tratamiento farmacológico , Glucósidos/administración & dosificación , Liraglutida/administración & dosificación , Síndrome de Prader-Willi/tratamiento farmacológico , Adolescente , Compuestos de Bencidrilo/farmacología , Glucemia/efectos de los fármacos , Glucemia/metabolismo , Niño , Diabetes Mellitus/sangre , Diabetes Mellitus/etiología , Quimioterapia Combinada , Femenino , Péptido 1 Similar al Glucagón/agonistas , Glucósidos/farmacología , Humanos , Liraglutida/farmacología , Síndrome de Prader-Willi/sangre , Síndrome de Prader-Willi/complicaciones , Inhibidores del Cotransportador de Sodio-Glucosa 2/administración & dosificación , Inhibidores del Cotransportador de Sodio-Glucosa 2/farmacología , Resultado del Tratamiento
2.
Arch Dis Child ; 102(6): 543-549, 2017 06.
Artículo en Inglés | MEDLINE | ID: mdl-28137708

RESUMEN

OBJECTIVE: To determine accuracy, safety and acceptability of the FreeStyle Libre Flash Glucose Monitoring System in the paediatric population. DESIGN, SETTING AND PATIENTS: Eighty-nine study participants, aged 4-17 years, with type 1 diabetes were enrolled across 9 diabetes centres in the UK. A factory calibrated sensor was inserted on the back of the upper arm and used for up to 14 days. Sensor glucose measurements were compared with capillary blood glucose (BG) measurements. Sensor results were masked to participants. RESULTS: Clinical accuracy of sensor results versus BG results was demonstrated, with 83.8% of results in zone A and 99.4% of results in zones A and B of the consensus error grid. Overall mean absolute relative difference (MARD) was 13.9%. Sensor accuracy was unaffected by patient factors such as age, body weight, sex, method of insulin administration or time of use (day vs night). Participants were in the target glucose range (3.9-10.0 mmol/L) ∼50% of the time (mean 12.1 hours/day), with an average of 2.2 hours/day and 9.5 hours/day in hypoglycaemia and hyperglycaemia, respectively. Sensor application, wear/use of the device and comparison to self-monitoring of blood glucose were rated favourably by most participants/caregivers (84.3-100%). Five device related adverse events were reported across a range of participant ages. CONCLUSIONS: Accuracy, safety and user acceptability of the FreeStyle Libre System were demonstrated for the paediatric population. Accuracy of the system was unaffected by subject characteristics, making it suitable for a broad range of children and young people with diabetes. TRIAL REGISTRATION NUMBER: NCT02388815.


Asunto(s)
Automonitorización de la Glucosa Sanguínea/métodos , Glucemia/análisis , Diabetes Mellitus Tipo 1/sangre , Adolescente , Automonitorización de la Glucosa Sanguínea/efectos adversos , Automonitorización de la Glucosa Sanguínea/instrumentación , Automonitorización de la Glucosa Sanguínea/normas , Niño , Preescolar , Relojes Circadianos/fisiología , Diabetes Mellitus Tipo 1/tratamiento farmacológico , Esquema de Medicación , Femenino , Hemoglobina Glucada/metabolismo , Humanos , Hipoglucemiantes/administración & dosificación , Insulina/administración & dosificación , Masculino , Aceptación de la Atención de Salud
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