ABSTRACT
Los avances en el campo de la fibrosis quística han aumentado la esperanza de vida de estos pacientes, por lo que cada vez es más prevalente la Diabetes Relacionada con la Fibrosis Quística (DRFQ) y sus complicaciones. La DRFQ se asocia a mayor morbimortalidad, deterioro de la función pulmonar y del estado nutricional. Por lo mismo, el manejo óptimo de esta patología depende de un diagnóstico precoz, tratamiento individualizado y vigilancia de las complicaciones diabéticas. El screening de DRFQ debe realizarse anualmente a partir de los 10 años, mediante una Prueba de Tolerancia a la Glucosa Oral (PTGO), lo cual permite el diagnóstico. El manejo de esta patología tiene por objetivo estabilizar y mejorar la función pulmonar y el estado nutricional y metabólico de los pacientes. Actualmente, la insulina es el tratamiento farmacológico de elección para controlar la hiperglicemia y el esquema de uso debe ser individualizado para cada persona. En caso de enfermedades agudas pueden existir mayores requerimientos de insulina. Además, se deben tener consideraciones especiales en cuanto a la dieta y la insuficiencia pancreática exocrina que presentan estos pacientes. Para la vigilancia de complicaciones microvasculares se debe realizar una monitorización anual a partir de los 5 años desde el diagnóstico de DRFQ. Debido a la complejidad de estos pacientes, para alcanzar el mejor cuidado posible se necesita un enfoque multidisciplinario con distintos profesionales de la salud coordinados, incluyendo en la toma de decisiones al paciente y su familia.
Advances made in the field of cystic fibrosis have increased the life expectancy of these patients, which is why Cystic Fibrosis-Related Diabetes (CFRD) and its complications are becoming more and more prevalent. CFRD is associated with increased morbidity and mortality, lower lung function and inadequate weight maintenance. Therefore, the optimal management of this pathology depends on an early diagnosis, individualized treatment and monitoring of diabetic complications. For CFRD, routine screening with an Oral Glucose Tolerance Test (OGTT) should be carried out yearly from the age of 10, which allows to diagnose it. The treatment goals in CFRD are to stabilize and improve lung function and obtain adequate weight gain. Currently, insulin is the pharmacological treatment of choice to control hyperglycemia and the insulin regimen must be personalized for each person. In acute illnesses, there may be higher insulin requirements. In addition, special considerations must be taken regarding diet and exocrine pancreatic insufficiency that these patients present. For the surveillance of microvascular complications, annual monitoring should be carried out 5 years after the diagnosis of CFRD. Due to the complexity of these patients, in order to achieve the best possible care, a multidisciplinary approach is needed with different coordinated health professionals, including the patients and their family in the decision-making process.
Subject(s)
Humans , Cystic Fibrosis/complications , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Diabetes Mellitus/etiology , Diabetes Mellitus/therapy , Patient Care Team , Mass Screening , Cystic Fibrosis/physiopathology , Nutrition Therapy , Diabetes Mellitus/diagnosis , Diabetes Mellitus/physiopathology , Insulins/therapeutic use , Glucose Tolerance Test , Hypoglycemic Agents/therapeutic useABSTRACT
OBJECTIVE:To investigate the effect of insulin use intervention and self management of blood glucose in the treat-ment of cystic fibrosis related diabetes. METHODS:In prospective cohort study,during Apr. 2007-Apr. 2013,1 295 CFRD inpa-tients selected from 14 hospitals of Shanghai were randomly divided into trial group (743 cases) and control group (552 cases). Control group maintained the dose of insulin,and trial group adjusted the dose of insulin every week and received patient education about self management of blood glucose. Blood glucose,blood lipid,quality of life,the ability of self management and other in-dex were detected,recorded and compared between 2 groups 1 year after intervention. RESULTS:1 year after intervention,fasting blood glucose,postprandial 2 h blood glucose and HbA1c of trial group were significantly lower than those of control gorup,with statistical significance(P<0.05). The time of blood glucose reaching the standard,the dose of insulin,the number of hyperglycemia and hypoglycemia in trial group were all significantly shorter or lower than control group,with statistical significance (P<0.05). There was statistically significant difference in the rate of standard blood glucose between trial group [93.5%(695/743)] and control group [59.6%(329/552)]. TG of trial group decreased while HDL increased,there was statistically significant difference between 2 groups (P<0.05). There was statistically significant difference in the quality of life score between trial group (196.24 ± 15.80) and control group(180.33±16.29)(P<0.05). The ability of self management in trial group was significantly higher than control group, with statistical significance(P<0.05). CONCLUSIONS:Insulin use intervention and self management of blood glucose can control the level of blood glucose in CFRD patients,and improve the quality of life,medication compliance and therapeutic effect.
ABSTRACT
Cystic fibrosis (CF) is the most common recessive autosomal disease among Caucasian. Children with CF have benefitted from advances in medical and nutritional treatments, and this can be gleaned from the improvement in the survival of these patients. The increase in the survival rate brought with it the appearance of co-morbidities related to CF. Nowadays cystic fibrosis-related diabetes (CFRD) is considered the most common complication associated with CF. It can appear as early as infancy or adolescence, and its prevalence can be as high as 50 percent in adult patients. Because of its high prevalence, difficulties in early detection and the risks involved, in recent years several studies and consensuses have focused on this condition, adding information about the epidemiology, pathophysiology, prognosis and treatment of CFRD. The main aspects of these new concepts, as well as the current recommendations for its diagnosis and follow-up, will be presented in this study.
Fibrose cística (FC) é a doença autossômica recessiva mais comum nos caucasianos. Avanços no tratamento da FC acarretaram aumento na sobrevida dos pacientes, que trouxe o aparecimento de comorbidades relacionadas à doença. Atualmente, o diabetes relacionado à fibrose cística (DRFC) é considerado a complicação mais comum associada à FC, podendo aparecer já na infância ou adolescência, chegando a atingir até 50 por cento dos pacientes adultos. Em virtude da alta prevalência, das dificuldades de diagnosticar precocemente e das graves consequências para os pacientes, nos últimos anos vem crescendo a preocupação com a detecção e o tratamento precoces do DRFC. Vários grupos têm se dedicado a procurar evidências e desenvolver consensos com o objetivo de orientar o diagnóstico e o acompanhamento dessa comorbidade. Neste artigo apresentamos os principais aspectos dessa evolução, bem como as atuais recomendações no acompanhamento de pacientes com DRFC.
Subject(s)
Adolescent , Adult , Child , Humans , Cystic Fibrosis/complications , Diabetes Mellitus/diagnosis , Cystic Fibrosis/epidemiology , Diabetes Mellitus/epidemiology , Diabetes Mellitus/etiology , Early DiagnosisABSTRACT
O diabetes melito (DM) é a principal comorbidade relacionada à fibrose cística (FC) e costuma acontecer após os 15 anos de idade, associando-se ao aumento da morbimortalidade. Sua fisiopatologia inclui destruição do tecido exócrino, insuficiência endócrina e aumento da resistência insulínica, determinando inicialmente a alteração da cinética secretora da insulina, até o comprometimento de sua secreção total. A hiperglicemia pós-prandial é a alteração clínica mais precoce e sintomas do DM podem não ser reconhecidos. A investigação anual deve ser realizada por meio da glicemia de jejum ou de glicemias ao acaso e do teste oral de tolerância à glicose (OGTT). São reconhecidas duas categorias de diabetes relacionadas à FC: o DRFC sem hiperglicemia de jejum (HJ) (glicemia de jejum < 126 mg/dL e glicemia de 2 horas OGTT > 200 mg/dL) e DRFC com HJ (glicemia de jejum > 126 mg/dL). O tratamento inclui o manejo nutricional especializado e a correção da hiperglicemia. A insulinoterapia é recomendada para a categoria DRFC com HJ, não existindo ainda evidências do benefício de drogas secretagogas ou sensibilizantes da ação insulínica.
Cystic fibrosis-related diabetes (CFRD) is a major co-morbidity generally affecting patients over 15 years old and it is associated with increased morbidity and mortality. The pathophysiology includes exocrine tissue destruction, insulin deficiency and insulin resistance; the carbohydrate metabolism dysfunction begins with an altered kinetic in insulin secretion followed by a progressive insulin deficiency. Postprandial hyperglycemia is the first abnormality seen in CF patients and the classical symptoms of diabetes may not be recognized. The screening strategy proposed is annual random plasma glucose or fasting plasma glucose investigation, as well as the performance the oral glucose tolerance test (OGTT). Two categories of diabetes are related to CF: CFRD without fasting hyperglycemia (fasting glucose < 126 mg/dL and 2h OGTT glucose > 200 mg/dL) and CFRD with fasting hyperglycemia (fasting glucose > 126 mg/dL). Nutritional management and hyperglycemia control are the CFRD treatment goals. Insulin control is the standard medical therapy for CFRD with fasting hyperglycemia and the benefits of oral insulin secretagogue and sensitizing agents are still controversial.