China
Africa
Explore chapters and articles related to this topic
Gene Therapy and Small Molecules Used in the Treatment of Cystic Fibrosis
Published in Yashwant Pathak, Gene Delivery, 2022
Manish P. Patel, Uma G. Daryai, Mansi N. Athalye, Praful D. Bharadia, Jayvadan Patel
Patients with cystic fibrosis related diabetes are observed to have symptoms as unexplained polyuria, failure to maintain weight, delayed progression of puberty, and unexplained chronic decline in pulmonary function (O’Riordan et al., 2009).
Endocrine Disorders
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Lisa Spence, Nana Adwoa Gletsu Miller, Tamara S. Hannon
Endocrine hormones are chemical signals that exert effects on tissue and organs throughout the body. Hormones play a critical role in the regulation of physiological homeostasis and are involved in growth and development, appetite regulation, metabolism, sexual and reproductive function, sleep, and mental health. Endocrine diseases result from abnormal levels of endocrine hormones or abnormalities in the signaling response. Diabetes mellitus, which occurs when insulin is not produced or when insulin signaling is impaired, is a common endocrine disease in children. Diabetes mellitus include type 1 diabetes, type 2 diabetes, and prediabetes. Related diseases include cystic fibrosis-related diabetes and steroid-induced hyperglycemia. Diabetes insipidus results from a disorder in the production or action of vasopressin. Youth with gender dysphoria may have higher rates of overweight and obesity. Growth hormone deficiency in children can lead to short stature, but in most cases the cause of short stature is constitutional, secondary to genetics or to nutrition, as well as to complications of non-endocrine diseases. Nutrition is an important consideration for clinical management of endocrine diseases as diet may play a role in the etiology of the disease or diet may modify the response to therapy. Moreover, nutrition status may be used as a criterion for diagnosis of the disease or to evaluate its progression. The objective of this chapter is to describe the pathophysiology, medical management, and medical nutrition therapy of common pediatric endocrine disorders.
Disorders of the respiratory system
Published in Judy Bothamley, Maureen Boyle, Medical Conditions Affecting Pregnancy and Childbirth, 2020
Many women with CF who are not diabetic before pregnancy may have impaired glucose tolerance38, so early (and perhaps repeated) screening for gestational diabetes should be done. It is common for these women to need insulin during pregnancy43. A woman with cystic fibrosis related diabetes (CFRD) or a new diagnosis of gestational diabetes will need advice from a specialist CF dietitian, as a high-fat high-energy diet is necessary in CF38 and this is contrary to normal advice around diabetes.
Pharmacological management of cystic fibrosis related diabetes
Published in Expert Review of Clinical Pharmacology, 2018
Cystic fibrosis related diabetes (CFRD) is associated with worsening of nutritional status and pulmonary function in patients with cystic fibrosis (CF).Progressive insulin insufficiency is the primary etiology.Current clinical practice guidelines of Cystic Fibrosis Foundation recommend insulin as the only pharmacological therapy for CFRD.Insulin therapy improves glycemic control, nutritional status and lung outcomes in patients with CFRD.Oral hypoglycemic agents are not currently recommended for treatment of CFRD
Mental health care needs in cystic fibrosis: A scoping review
Published in Social Work in Health Care, 2022
Campbell Tickner, Lucy Holland, Peter Wark, Jodi Hilton, Cathy Morrison, Frances Kay-Lambkin, Milena Heinsch
Cystic fibrosis is a significantly life-limiting inherited genetic disease that causes long-lasting lung infections and limits the ability to breathe over time (Cystic Fibrosis Foundation, n.d.). Despite tremendous medical advancements, cystic fibrosis continues to be one of the most challenging and burdensome chronic illnesses (Smith et al., 2016). While the symptoms and severity of cystic fibrosis vary, the disease is commonly characterized by chronic lung infection, pancreatic insufficiency, and male infertility, and can lead to comorbidities such as cystic fibrosis-related diabetes and liver disease (Shteinberg et al., 2021). People affected by this condition experience declining health and function over time and, despite remarkable advancements in care, undergo diverse, highly burdensome, and time-consuming multimodal treatment regimens (Smith et al., 2016). Further, people with cystic fibrosis are subjected to stringent infection control guidelines, which prohibit face-to-face contact with other individuals who have cystic fibrosis. These factors can lead to significant psychological distress and social isolation for people with cystic fibrosis, factors that have been exacerbated by the COVID-19 pandemic due to uncertainty about the impact of the virus on people with cystic fibrosis and the stringent isolation protocols in place (Chen et al., 2021; Westcott et al., 2021). Importantly, psychological symptoms in people with cystic fibrosis have been found to be associated with decreased lung function, lower body mass index and reduced adherence to disease-related treatment, worse health-related quality of life, more frequent hospitalizations, and increased healthcare costs (Quittner et al., 2016).