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1.
Clin Endocrinol (Oxf) ; 91(6): 770-775, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31520536

RESUMO

OBJECTIVE: Diazoxide is first-line treatment for hyperinsulinaemic hypoglycaemia (HH) but diazoxide-induced pulmonary hypertension (PH) can occur. We aim to characterize the incidence and risk factors of diazoxide-induced PH in a large HH cohort to provide recommendations for anticipating and preventing PH in diazoxide-treated patients with HH. DESIGN AND PATIENTS: Retrospective cohort study involving four UK regional HH centres; review of case notes of HH patients on diazoxide. MEASUREMENTS: The diagnosis of PH was based on clinical and echocardiography evidence. Patient and treatment-related risk factors were analysed for association. RESULTS: Thirteen (6 men) of 177 HH diazoxide-treated patients developed PH, an incidence of 7%. In the PH group, HH was diagnosed at median (range) of 9 (1,180) days, with diazoxide commenced 4 (0,76) days from diagnosis and reaching a maximum dose of 7 (2.5,20) mg/kg/d. The majority (8 of 13 patients) developed PH within 2 weeks of diazoxide. Complete diazoxide withdrawal, but not dose reduction, led to PH resolution at 41 (3,959) days. In three patients, PH continued beyond 12 months. Risk factors for the development of PH included the presence of congenital heart disease (CHD) (P = .008), and total fluid volume exceeding 130 mL/kg/d in the immediate 24 hours preceding diazoxide (P = .019). CONCLUSION: Pulmonary hypertension can occur in 7% of diazoxide-treated HH patients. Risk factors include the presence of congenital heart disease and fluid overload. Recommendations include echocardiography and fluid restriction to 130 mL/kg/d prior to diazoxide treatment and immediate discontinuation of diazoxide if PH develops.


Assuntos
Hiperinsulinismo Congênito/tratamento farmacológico , Hiperinsulinismo Congênito/fisiopatologia , Diazóxido/efeitos adversos , Diazóxido/uso terapêutico , Hipertensão Pulmonar/induzido quimicamente , Hipoglicemia/fisiopatologia , Hiperinsulinismo Congênito/genética , Ecocardiografia , Feminino , Idade Gestacional , Humanos , Hipertensão Pulmonar/genética , Hipoglicemia/genética , Masculino , Canais de Potássio Corretores do Fluxo de Internalização/genética , Estudos Retrospectivos , Fatores de Risco , Receptores de Sulfonilureias/genética , Reino Unido
2.
Diabetes Metab Syndr ; 13(5): 2923-2926, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31425957

RESUMO

BACKGROUND: In 2017 the UK's Association of Children's Diabetes Clinicians (ACDC) launched a national educational package to provide training in the use of Freestyle Flash glucose monitoring (GM) to healthcare professionals. OBJECTIVE: To evaluate metabolic outcomes and quality of life (QoL) of children with T1DM trained in the use of the Freestyle Flash GM system adopting the ACDC guidelines. METHODS: Prospective study conducted at a single UK children's diabetes unit from 2017 to 2018.52 children with T1DM (age 5-18 yrs) were commenced on the Freestyle Flash GM system, received education and were followed up for 12 months. The Peds QL 3.2 diabetes questionnaire was used to assess QoL before and after the use of the system. HbA1c was measured at 3, 6 and 12 months pre and post use of Freestyle. RESULTS: 52 children (33 M,19 F) with a mean age of 11.6 yrs (range 4 m-17.2 yrs) were evaluated. Mean HbA1c 3 months post Freestyle Flash GM showed a significant improvement when compared with HbA1c values at 12, 6 and 3 months pre Freestyle (p-value 0.040, 0.040, 0.012 respectively). This improvement was not sustained at 6 and 12 months (p-value 0.15, 0.50). The PedsQL3.2 diabetes scores demonstrated significant improvement in patient QoL, reduction of diabetes symptoms and treatment barriers following the use of the new technology (p-values 0.014; 0.018; 0.035 respectively). CONCLUSIONS: Freestyle Flash GM technology associated with appropriate education and regular support by healthcare professionals improves patient quality of life measures in children with T1DM.


Assuntos
Automonitorização da Glicemia/instrumentação , Glicemia/análise , Diabetes Mellitus Tipo 1/sangue , Diabetes Mellitus Tipo 1/prevenção & controle , Pessoal de Saúde/estatística & dados numéricos , Educação de Pacientes como Assunto , Qualidade de Vida , Adolescente , Biomarcadores/análise , Criança , Pré-Escolar , Diabetes Mellitus Tipo 1/epidemiologia , Feminino , Seguimentos , Humanos , Hipoglicemiantes/uso terapêutico , Lactente , Masculino , Prognóstico , Estudos Prospectivos , Reino Unido/epidemiologia
3.
J Clin Res Pediatr Endocrinol ; 11(1): 104-108, 2019 02 20.
Artigo em Inglês | MEDLINE | ID: mdl-30154070

RESUMO

Recent studies have shown that small for gestational age (SGA) term infants undergo catch-up growth during infancy but there is limited studies on early growth outcomes of extreme premature SGA infants. The aim of this study was to compare factors associated during birth in extremely premature infants less than 28 weeks' gestation who were born SGA (<10th percentile for gestational age) with those who were born appropriate-for-gestational age (AGA) (10th-89th percentile) and to determine whether there was catch-up growth at term equivalence. One hundred fifty-three extreme premature infants (89 males) born below 28 weeks' gestation were prospectively recruited. All infants had auxological measurements undertaken and prospective data on pregnancy, maternal factors, perinatal and postnatal data obtained. SGA infants at birth had significantly higher Clinical Risk Index for Babies scores and mortality, lower birth weight, smaller head circumference, smaller mid arm circumference and shorter leg length at time of birth compared with AGA infants. However, at term equivalence, weight and leg length of were not significant between AGA and SGA infants born at extreme prematurity. Our study shows that extreme premature SGA infants have appropriate catch-up growth by the time they reach term equivalence suggesting that postnatal nutrition and care are important determinants of catch-up growth in SGA infants.


Assuntos
Peso ao Nascer/fisiologia , Recém-Nascido Prematuro/crescimento & desenvolvimento , Recém-Nascido Pequeno para a Idade Gestacional/crescimento & desenvolvimento , Recém-Nascido de muito Baixo Peso/crescimento & desenvolvimento , Antropometria/instrumentação , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Perna (Membro)/anatomia & histologia , Perna (Membro)/crescimento & desenvolvimento , Masculino , Estudos Prospectivos
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