Cystic fibrosis-related diabetes

Author(s):  
Stephen MP O’Riordan ◽  
Antoinette Moran

This chapter on CFRD reviews the ever-evolving topic and provides up-to-date information on how to diagnose and manage cystic fibrosis-related diabetes CFRD in the acute and chronic setting. The treatments necessary to treat and prolong life in CF, including their unique dietary requirements, must always be followed as a first priority, with diabetes care adjusted accordingly. Early intervention with insulin has been shown to reverse clinical deterioration, even in those with mild diabetes. Newly emerging treatments for CF which have the potential to restore defective chloride channels may have implications for the development and treatment of CFRD. Whilst CFRD shares features of both type 1 and type 2 diabetes, there are important differences which necessitate a unique approach to diagnosis and management. Factors specific to CF that variably affect glucose metabolism include chronic respiratory infection and inflammation, increased energy expenditure, malnutrition, glucagon deficiency, and gastrointestinal abnormalities.

2019 ◽  
Author(s):  
Ildiko Lingvay ◽  
Philip Raskin

Secondary forms of diabetes mellitus are those cases of diabetes mellitus that have a specific identifiable cause and do not meet the diagnostic criteria for type 1, type 2, or gestational diabetes. This review discusses the etiology, pathogenesis, diagnosis, management, complications, and prognosis of these forms, which include diabetes mellitus occurring as a result of pancreatic disorders; endocrinopathies; drugs, chemical agents, or toxins; and genetic mutations or syndromes. Tables list the endocrinopathies; the drug, chemicals, and toxins; and the genetic disorders causing secondary forms of diabetes mellitus. This review contains 3 tables and 15 references. KeyWords: chronic pancreatitis, pancreatic carcinoma, cystic fibrosis, hemochromatosis, malnutrition, diabetic ketoacidosis or symptomatic hyperglycemia or hypoglycemia


2013 ◽  
pp. n/a-n/a ◽  
Author(s):  
Katja Konrad ◽  
Nicole Scheuing ◽  
Klaus Badenhoop ◽  
Martin H. Borkenstein ◽  
Bettina Gohlke ◽  
...  

Hypertension ◽  
2021 ◽  
Vol 78 (Suppl_1) ◽  
Author(s):  
Kirthikaa Balapattabi ◽  
Gary Mouradian ◽  
Matthew Hodges ◽  
Curt D Sigmund ◽  
Justin L Grobe

The adverse cardiovascular responses to leptin are preserved, but beneficial metabolic effects, such asincreases in resting metabolic rate (RMR) are lost in obesity induced by high fat diet (HFD) feeding. Wepreviously reported angiotensin II (ANG) type 1a ( Agtr1a ; AT1A) receptors in a subset of AgRP neurons arecritically required for the integrative control of RMR. Understanding the mechanisms in this unique subset ofRMR controlling- AgRP neurons is the goal of this study and is critical to decipher the pathogenesis of obesity-associated hypertension. Male C57BL/6J mice were maintained on chow or HFD from 8-18 weeks of age. Cell-attached voltage clamp recording of AgRP neurons in ARC sections of Ai9(tdTomato) AgRP mice showed that ANGapplication results in three distinct cellular responses (n=65 neurons; 8 mice). In chow fed mice, ANG suppressedelectrical activity in 1/3 of AgRP neurons (ie. “Type 1” AgRP neurons) via a losartan-sensitive mechanism,indicating involvement of AT1 receptors (Firing rate; aCSF:1.23 ± 0.10 vs ANG:0.60 ± 0.08 vs ANG+LOS:1.37 ±0.11 Hz, p<0.05). ANG caused excitation in 1/3 of AgRP neurons (“Type 2” AgRP cells) which was mediatedthrough a PD-123,319-sensitive mechanism, implicating AT2 receptors (ANG:1.69 ± 0.12 vs ANG+PD:0.86 ±0.06 Hz, p<0.05). Finally, 1/3 of AgRP neurons did not respond to ANG (“Type 0” cells). Complementary to thesefindings, mice with AT1A deleted from AgRP cells (ie, Agrp -Cre x Agtr1a Flox x Ai9 mice) exhibited a completeloss of Type 1 responses but maintenance of Types 0 and 2 (n=23 cells; 4 mice). Further, pharmacologicaldissection of signaling cascades implicated a pertussis toxin-sensitive mechanism (Gαi cascade; ANG+PTX:1.00± 0.04 Hz, p<0.05) and multiple potassium + chloride channels in the ANG-mediated inhibition of Type 1 cells.Most intriguingly, the relative proportion of AgRP neurons exhibiting Type 1 (ANG -inhibited) vs Type 0 or 2responses was decreased with HFD (Type 1 cells proportion- chow: 35%,23 out of 65; HFD: 18%, 10 out of 56).These results establish a specific ANG-inhibited subtype of AgRP neuron and implicate the AT1A/Gαi signalingcascade in the inhibitory effect of ANG. Switch of Type 1 AgRP cells to Type 0 or 2 in response to HFD suggestsHFD-induced neural plasticity/adaptation.


2019 ◽  
Author(s):  
Ildiko Lingvay ◽  
Philip Raskin

Secondary forms of diabetes mellitus are those cases of diabetes mellitus that have a specific identifiable cause and do not meet the diagnostic criteria for type 1, type 2, or gestational diabetes. This review discusses the etiology, pathogenesis, diagnosis, management, complications, and prognosis of these forms, which include diabetes mellitus occurring as a result of pancreatic disorders; endocrinopathies; drugs, chemical agents, or toxins; and genetic mutations or syndromes. Tables list the endocrinopathies; the drug, chemicals, and toxins; and the genetic disorders causing secondary forms of diabetes mellitus. This review contains 3 tables and 15 references. KeyWords: chronic pancreatitis, pancreatic carcinoma, cystic fibrosis, hemochromatosis, malnutrition, diabetic ketoacidosis or symptomatic hyperglycemia or hypoglycemia


Diabetes ◽  
2018 ◽  
Vol 67 (Supplement 1) ◽  
pp. 1521-P
Author(s):  
CHRISTINE L. CHAN ◽  
ANDREA STECK ◽  
TIM B. VIGERS ◽  
LAURA PYLE ◽  
FRAN DONG ◽  
...  

2020 ◽  
Vol 4 (1-3) ◽  
pp. 5
Author(s):  
Santiago Herrero

Anyone is at risk of contracting COVID-19 if exposed to the virus. Some people are more likely to become seriously ill than others, which means they may need hospitalization, intensive care, or respiratory support (respirator mask, high flow oxygen therapy, ventilators, etc.), and some may even die, mainly by severe respiratory failure. Those patients with asthma (moderate to severe), cardiovascular disease, cystic fibrosis, hypertension, immunosuppression, liver disease, pregnancy, smoker, thalassemia and diabetes (especially type 1) have a higher risk of getting sick and suffering the disease with more virulence and potential mortality. The older people are in risk because the chronical diseases and comorbidities are associate with the aging.This presentation aims to explain the mechanisms of infection and inflammation by the coronavirus in order to act primarily on them. If you know your enemy well, you can treat it from an etiopathogenic perspective.


2018 ◽  
Vol 104 (9) ◽  
pp. 887-889 ◽  
Author(s):  
Hoong-Wei Gan ◽  
Jayesh Mahendra Bhatt ◽  
Louise Denvir ◽  
Tabitha Randell ◽  
Pooja Sachdev

We present a non-consanguineous family of three siblings who presented with diabetes mellitus (DM), two of whom had genetically confirmed cystic fibrosis (CF), with one pancreatic-sufficient mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene (ΔF508/R117H;IVS8-5T). A detailed history revealed family members from three successive generations diagnosed with ‘type 1’ or ‘type 2’ diabetes, leading to genetic investigations for monogenic DM. A heterozygous frameshift mutation in the hepatocyte nuclear factor 1 homeobox alpha (HNF1A) gene (c.404delA) was subsequently confirmed in all three siblings, which is known to cause monogenic diabetes and is exquisitely sensitive to sulfonylurea therapy. Following this diagnosis, both siblings with CF and HNF1A monogenic diabetes were started on gliclazide therapy, while their older brother who had been wrongly diagnosed with type 1 diabetes was switched from insulin to gliclazide, all with excellent therapeutic responses.


2019 ◽  
Vol 2 (1) ◽  
pp. 23-27
Author(s):  
Liviu-Laurențiu Pop ◽  
Mihaela Dediu ◽  
Iulian Velea ◽  
Mirela Mogoi ◽  
Ioana M. Ciuca

AbstractCystic fibrosis related diabetes (CFRD) is a redoubtable complication associated to cystic fibrosis, with an increasing frequency, directly proportional to children life expectancy. Although this complication has similar features with DM type 1 and some with type 2, the evolution and even the response to insulin therapy is different. It is also possible that other factors to influence the CFRD clinical expression and subsequently the disease evolution. Since its 1t diagnosis was associated with more frequent pulmonary exacerbations and with the deterioration of the respiratory status, therefore CFRD must be early and correctly diagnosed and managed. The aim of this paper is to present an overview of the recent updates and recommendations regarding this important CF complication.


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