Hyperglycemia and Hypoinsulinemia in Patients with Fanconi-Bickel Syndrome

2008 ◽  
Vol 21 (6) ◽  
pp. 581-586
Author(s):  
Doris Taha ◽  
Naffaa Al-Harbi ◽  
Essam Al-Sabban
Keyword(s):  
Glucose Tolerance ◽  
Glucose Transporter ◽  
Serum Insulin ◽  
Autosomal Recessive Disorder ◽  
Tolerance Test ◽  
Oral Glucose ◽  
Glycogen Accumulation ◽  
Transporter Protein ◽  
Fasting Hypoglycemia ◽  
Insulin Responses

Abstract Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder characterized by the combination of hepatorenal glycogen accumulation and Fanconi-type nephropathy. Mutations in GLUT2, the gene for facilitative glucose transporter protein 2 (GLUT2), cause FBS. Aim: To evaluate glucose and insulin responses to oral glucose load in patients with FBS. Methods: Ten children (7.3 ± 4.8 years) diagnosed with FBS in early infancy underwent a standard oral glucose tolerance test (OGTT); plasma glucose (PG) and serum insulin concentrations were measured at 30-min intervals for 2 hours. HbAtc, insulin-like growth factor-1, and fasting lipid profiles were also measured. Results: Mean fasting and 2-h PG concentrations were 3.8 ± 0.9 mmoVI and 8.6 ± 3.0 mmol/1, respectively. 2-hour PG levels were above 11.1 mmolll in two patients (20%) and between 7.75 and 11.1 mmoVI in four patients (40%). HbAtc was normal in all the patients with a mean of 5.4 ± 0.3%. Mean fasting and peak serum insulin levels were 8. 7 ± 0.8 pmol/1 and 98.6 ± 43.0 pmoVI, respectively, and did not differ between / the patients with normal and abnormal OGTT. , Patients with abnormal OGTT were younger ( 4.8 ± 3.2 vs 11.0 ± 4.8 yr; p = 0.04). Fasting PG increased with age (r = 0.80, p <0.01). Total and LDL cholesterol as well as triglyceride concentrations were elevated. Conclusions: Most but not all patients with FBS have impaired glucose tolerance/diabetes range hyperglycemia after OGTT while maintaining normal HbAtc· Patients with FBS are relatively hypoinsulinemic. Both fasting hypoglycemia and post-OGTT hyperglycemia seem to improve with age.

scholarly journals Effect of breakfast fat content on glucose tolerance and risk factors of atherosclerosis and thrombosis

1998 ◽  
Vol 80 (4) ◽  
pp. 323-331 ◽  
Author(s):  
David L. Frape ◽  
Norman R. Williams ◽  
Jayshri Rajput-Williams ◽  
B. W. Maitland ◽  
A. J. Scriven ◽  
...  
Keyword(s):  
Glucose Tolerance ◽  
Thrombus Formation ◽  
Serum Insulin ◽  
Lipid Fraction ◽  
Tolerance Test ◽  
Serum Glucose ◽  
Oral Glucose ◽  
Significant Treatment ◽  
Treatment Allocation ◽  
Insulin Responses

Twenty-four middle-aged healthy men were given a low-fat high-carbohydrate (5.5 g fat; L), or a moderately-fatty, (25.7 g fat; M) breakfast of similar energy contents for 28 d. Other meals were under less control. An oral glucose tolerance test (OGTT) was given at 09.00 hours on day 1 before treatment allocation and at 13.30 hours on day 29. There were no significant treatment differences in fasting serum values, either on day 1 or at the termination of treatments on day 29. The following was observed on day 29: (1) the M breakfast led to higher OGTT C-peptide responses and higher areas under the curves (AUC) of OGTT serum glucose and insulin responses compared with the OGTT responses to the L breakfast (P< 0.05); (2) treatment M failed to prevent OGTT glycosuria, eliminated with treatment L; (3) serum non-esterified fatty acid (NEFA) AUC was 59% lower with treatment L than with treatment M, between 09.00 and 13.20 hours (P<0.0001), and lower with treatment L than with treatment M during the OGTT (P= 0.005); (4) serum triacylglycerol (TAG) concentrations were similar for both treatments, especially during the morning, but their origins were different during the afternoon OGTT when the Svedberg flotation unit 20–400 lipid fraction was higher with treatment L than with treatment M (P= 0.016); plasma apolipoprotein B-48 level with treatment M was not significantly greater than that with treatment L (P= 0.086); (5) plasma tissue plasminogen-activator activity increased after breakfast with treatment L (P= 0.0008), but not with treatment M (P= 0.80). Waist:hip circumference was positively correlated with serum insulin and glucose AUC and with fasting LDL-cholesterol. Waist:hip circumference and serum TAG and insulin AUC were correlated with factors of thrombus formation; and the OGTT NEFA and glucose AUC were correlated. A small difference in fat intake at breakfast has a large influence on circulating diurnal NEFA concentration, which it is concluded influences adversely glucose tolerance up to 6 h later.

Chronic exercise increases insulin binding in muscles but not liver

1986 ◽  
Vol 251 (2) ◽  
pp. E196-E203
Author(s):  
A. Bonen ◽  
P. A. Clune ◽  
M. H. Tan
Keyword(s):  
Glucose Tolerance ◽  
Plasma Membranes ◽  
Serum Insulin ◽  
Exercise Bout ◽  
Insulin Binding ◽  
Tolerance Test ◽  
Time Of Day ◽  
Oral Glucose ◽  
Chronic Exercise ◽  
Binding Data

It has been postulated that the improved glucose tolerance provoked by chronic exercise is primarily attributable to increased insulin binding in skeletal muscle. Therefore, we investigated the effects of progressively increased training (6 wk) on insulin binding by five hindlimb skeletal muscles and in liver. In the trained animals serum insulin levels at rest were lower either in a fed (P less than 0.05) or fasted (P less than 0.05) state and after an oral glucose tolerance test (n = 8) (P less than 0.05). Twenty-four hours after the last exercise bout sections of the liver, soleus (S), plantaris (P), extensor digitorum longus (EDL), and red (RG) and white gastrocnemius (WG) muscles were pooled from four to six rats. From control animals, killed at the same time of day, muscles and liver were also obtained. Insulin binding to plasma membranes increased in S, P, and EDL (P less than 0.05) but not in WG (P = 0.07), RG (P greater than 0.1), or in liver (P greater than 0.1). There were insulin binding differences among muscles (P less than 0.05). Comparison of rank orders of insulin binding data with published glucose transport data for the same muscles revealed that these parameters do not correspond well. In conclusion, insulin binding to muscle is shown to be heterogeneous and training can increase insulin binding to selected muscles but not liver.

Detection of Glycemic Abnormalities in Young Adult Patients with Beta Thalassemia Major Using Continuous Glucose Monitoring System (CGMS) and Oral Glucose Tolerance Test (OGTT)

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2158-2158
Author(s):  
Mohamed A. Yassin ◽  
Ahmed M Elawa ◽  
Ashraf T Soliman
Keyword(s):  
Glucose Tolerance ◽  
Oral Glucose Tolerance Test ◽  
Glucose Tolerance Test ◽  
Cell Function ◽  
Serum Insulin ◽  
Tolerance Test ◽  
Oral Glucose ◽  
Oral Glucose Tolerance ◽  
Β Cell ◽  
Β Cell Function

Abstract Abstract 2158 Introduction: Both insulin deficiency and insulin resistance are reported in patients with β thalassemia major (BTM). The use of continuous blood glucose monitoring system (CGMS) among the different methods for early detection of glycaemic abnormalities has not been studied thoroughly in these patients. Aims: The aims of this study were: 1. to detect glycaemic abnormalities, if any, in young adults with BTM using fasting blood glucose (FBG), oral glucose tolerance test (OGTT), 72-h continuous glucose concentration by CGMS system, and serum insulin and C-peptide concentrations 2. To compare the results of these two methods in detecting glycaemic abnormalities in these patients and 3. To calculate homeostatic model assessment (HOMA), and the quantitative insulin sensitivity check index (QUICKI) in these patients. In order to evaluate whether glycaemic abnormalities are due to insulin deficiency and/or resistance. Materials and methods: Randomly selected young adults (n = 14) with BTM were the subjects of this study. All patients were investigated using a standard oral glucose tolerance test (OGTT) (using 75 gram of glucose) and 72-h continuous glucose concentration by CGM system (Medtronic system). Fasting serum insulin and C-peptide concentrations were measured and HOMA-B, HOMA-IR were calculated accordingly. Results: Using OGTT, 5 patients had impaired fasting glucose (IFG) (Fasting BG from 5.6 to 6.9 mmol/L). Two of them had impaired glucose tolerance IGT (BG from 7.8 and < 11.1 mmol/L) and one had BG = 16.2 mmol/L after 2-hrs (diabetic). Using CGMS in addition to the glucose data measured by glucometer (3–5 times/ day), 6 patients had IFG. The maximum (postprandial) BG recorded exceeded 11.1 mmol/L in 4 patients (28.5%) (Diabetics) and was > 7.8 but < 11.1 mmol/L in 8 patients (57%) (IGT). The mean values of HOMA and QUICKI in patients with BTM were < 2.6 (1.6± 0.8) and > 0.33 (0.36±0.03) respectively ruling out significant insulin resistance in these adolescents. There was a significant negative correlation between the β-cell function (B %) on the one hand and the fasting and the 2-h BG (r= −0.6, and − 0.48, P< 0.01 respectively) on the other hand. Serum insulin concentrations were not correlated with fasting BG or ferritin levels. The average and maximum BG levels recorded by CGMS were significantly correlated with the fasting BG (r= 0.69 and 0.6 respectively with P < 0.01) and with the BG at 2-hour after oral glucose intake (r= 0.87and 0.86 respectively with P < 0.01). Ferritin concentrations were positively correlated with the fasting BG and the 2-h BG levels in the OGTT (r= 0.69, 0.43 respectively, P < 0.001) as well as with the average and the maximum BG recorded by CGM (r =0.75, and 0.64 respectively with P < 0.01). Ferritin concentrations were negatively correlated with the β-cell function (r= −0.41, P< 0.01). Conclusion: CGMS has proved to be superior to OGTT for the diagnosis of glycaemic abnormalities in young adult patients with BTM. In our patients, defective β-cell function rather than insulin resistance appeared to be the cause for these abnormalities. The significant correlations between serum ferritin concentrations and the beta cell functions suggested the importance of adequate chelation to prevent β-cell dysfunction Disclosures: No relevant conflicts of interest to declare.

scholarly journals Blackthorn Flower Extract Impact on Glycaemic Homeostasis in Normoglycemic and Alloxan-Induced Hyperglycaemic C57BL/6 Mice

2021 ◽  
Vol 59 (3) ◽  
Author(s):  
Irena Crnić ◽  
Tajana Frančić ◽  
Petar Dragičević ◽  
Vedran Balta ◽  
Verica Dragović-Uzelac ◽  
...  
Keyword(s):  
Blood Glucose ◽  
Glucose Tolerance ◽  
Glucose Homeostasis ◽  
Serum Insulin ◽  
Sugar Content ◽  
Tolerance Test ◽  
Oral Glucose ◽  
Mice Model ◽  
Flower Extract ◽  
Prunus Spinosa

Research background. The use of plants and their extracts in treatments of chronic diseases is widely known in traditional medicine. The aim of this study is to determine the effects of 10-day consumption of Prunus spinosa L. flower extract on blood glucose, glycaemic load, serum α-amlyase and serum insulin, in normoglycaemic and hypergycaemic (alloxan) mice model. Experimental approach. Normoglycemic and hyperglycemic (alloxan treated, 150 mg/kg body mass) C57BL/6 mice were treated daily, during 10 days, with Prunus spinosa L. flower extract by gavage. The sugar content within extract was determined by HPLC analysis. In mice, blood and serum blood glucose level and OGTT-test were determined by blood glucometer. Serum insulin was determined by ELISA assay and α-amlyase by colourimetric assay. Results and conclusions. The Prunus spinosa L. flower extract increased glucose in normoglycaemic mice by 30 % after 1st and 5th day and by 17 % after 10th day of consumption in normoglycaemic mice. It is a consequence of released sugars because sugar analysis revealed 59.8 mg/L monosaccharides, mainly fructose (55.7 mg/L) and glucose (24.3 mg/L) within the extract. On the opposite, the extract consumption, reduced serum blood glucose in alloxan-induced hyperglycaemic mice by 29 % after 10 days of treatment. Oral glucose tolerance test also confirmed that that in the hyperglycaemic group treated with Prunus spinosa L. flower extract glucose homeostasis was improved and showed decrease in blood glucose, since the blood glucose over the period of 120 min, glucose homeostasis is faster achieved after treatment with shows that in Prunus spinosa L. flower extract. Serum insulin increased by 49 % and serum alpha amylase by 46 % after 10 days of treatment with Prunus spinosa L. flower extract in hyperglycaemic group. Thus, it can be concluded that Prunus spinosa L. flower extract improved glucose tolerance, enhanced insulin secretion and lowered serum α-amylase activity. Novelty and scientific contribution. The results examined for the first time the potential of Prunus spinosa L. flower extract in hyperglycaemia management.

ABNORMAL INSULIN SECRETION IN PARKINSON'S DISEASE BEFORE AND DURING l-DIHYDROXYPHENYLALANINE (l-DOPA) THERAPY

1973 ◽  
Vol 59 (3) ◽  
pp. 523-534 ◽  
Author(s):  
M. H. VAN WOERT ◽  
P. S. MUELLER ◽  
L. M. AMBANI ◽  
U. RATHEY
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Glucose Tolerance ◽  
Serum Insulin ◽  
Chronically Ill ◽  
Control Group ◽  
Oral Glucose ◽  
Oral Glucose Tolerance ◽  
Intravenous Glucose ◽  
Insulin Responses

SUMMARY Insulin and glucose metabolism were studied in parkinsonian patients before and during treatment with l-DOPA and in a chronically ill, elderly, control group of patients. The parkinsonian patients had a low absolute glucose disappearance rate and a low serum insulin response to intravenous glucose compared with controls, which was not altered by l-DOPA therapy. The serum glucose and insulin responses to the oral glucose tolerance tests in parkinsonian patients were similar to those of the control group. Normal immediate insulin responses were observed after infusions of tolbutamide and glucagon. l-DOPA decreased serum insulin and glucose levels during the first 90 min of the oral glucose tolerance test and produced an increase in concentration of human growth hormone in serum of some parkinsonian patients. l-DOPA therapy had no effect on intravenous tolbutamide and glucagon tolerance tests. Our results indicate a selective defect in the mechanism of intravenous glucose-induced insulin release in patients with Parkinson's disease.

scholarly journals Obestatin Is Not Elevated or Correlated with Insulin in Children with Prader-Willi Syndrome

2007 ◽  
Vol 92 (1) ◽  
pp. 229-234 ◽  
Author(s):  
Won Hah Park ◽  
Yoo Joung Oh ◽  
Gae Young Kim ◽  
Sang Eun Kim ◽  
Kyung-Hoon Paik ◽  
...  
Keyword(s):  
Glucose Tolerance ◽  
Oral Glucose Tolerance Test ◽  
Glucose Tolerance Test ◽  
Medical Center ◽  
Serum Insulin ◽  
Tolerance Test ◽  
Oral Glucose ◽  
Prader Willi Syndrome ◽  
Oral Glucose Tolerance ◽  
Significant Difference

Abstract Context: Obestatin is a peptide hormone derived from the proteolytic cleavage of ghrelin preprohormone. In Prader-Willi syndrome (PWS), the levels of total ghrelin (TG) and acylated ghrelin (AG) are increased, and these hormones are regulated by insulin. Objective: Our objective was to analyze the changes in the obestatin levels after glucose loading and to characterize the correlations of obestatin with TG, AG, and insulin. Design: Plasma obestatin, TG, AG, and insulin levels were measured in PWS children (n = 15) and controls (n = 18) during an oral glucose tolerance test. Setting: All subjects were admitted to the Samsung Medical Center. Interventions: An oral glucose tolerance test was performed after an overnight fast. Main Outcome Measures: The plasma levels of obestatin, TG, AG, and serum insulin were measured at 0, 30, 60, 90, and 120 min after glucose challenge, and areas under the curves (AUCs) were calculated. Results: No significant difference in AUC of the plasma obestatin was found between the PWS children and normal obese controls (P = 0.885), although AUC of AG (P = 0.002) and TG (P = 0.003) were increased in the PWS children. Moreover, There was a negative correlation between the AUC of AG and AUC of insulin both in PWS (r = −0.432; P = 0.049) and in controls (r = −0.507; P = 0.016). However, AUC of obestatin was not significantly correlated with AUC of insulin (in PWS, r = 0.168 and P = 0.275; in controls, r = −0.331 and P = 0.09). Conclusions: Our results indicate that plasma obestatin is not elevated in PWS children and is not regulated by insulin both in PWS children and in obese controls.

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