Type I glycogen storage disease: favourable outcome on a strict management regimen avoiding increased lactate production during childhood and adolescence

D�ublin, Gerhard; Wendel, U.; Schwahn, Bernd

doi:10.1007/s00431-002-1001-1

Cited by 14 publications

(6 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Concerning the profile of the patients' nutritional status in the present analysis, the results differed from the study conducted by Däublin et al, 18 in which 23 patients with GSD I followed a restrictive diet and none of them were obese. In the research carried out by Schwahn et al, 19 only one out of 19 patients was obese, and patients with type Ia had lower weight compared with patients of type Ib, which was not observed in this study.…”

Section: Discussioncontrasting

confidence: 99%

“…According to some analyses, the initiation of diet therapy and the good metabolic control of the disease enable an accelerated growth, whereas untreated patients have slow growth. In addition, both weight and height can be close to the 50 th percentile; 18,23,24 nevertheless, Daeschel et al 24 emphasized that, when considering weight-for-height, patients were overweight. In a case study, Karnsakul et al 25 managed to revert short stature in a patient with GSD Ia, who even reached his target height by adequate metabolic control and diet therapy, but this resulted in obesity.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Anthropometric and Dietary Assessment of Patients With Glycogenosis Type I

Jorge

Tommaso

Hessel

2021

Rev. paul. pediatr.

View full text Add to dashboard Cite

Objective: To perform anthropometric and dietary evaluation of patients with glycogenosis type Ia and Ib. Methods: This cross-sectional study is composed of a sample of 11 patients with glycogenosis divided into two subgroups according to the classification of glycogenosis (type Ia=5 and type Ib=6), aged between 4 and 20 years. The analyzed anthropometric variables were weight, height, body mass index, and measures of lean and fat body mass, which were compared with reference values. For dietary assessment, a food frequency questionnaire was used to calculate energy and macronutrients intake as well as the amount of raw cornstarch consumed. Mann-Whitney U test and Fisher’s exact test were performed, considering a significance level of 5%. Results: Patients ingested raw cornstarch in the amount of 0.49 to 1.34 g/kg/dose at a frequency of six times a day, which is lower than recommended (1.75-2.50 g/kg/dose, four times a day). The amount of energy intake was, on average, 50% higher than energy requirements; however, carbohydrate intake was below the adequacy percentage in 5/11 patients. Short stature was found in 4/10 patients; obesity, in 3/11; and muscle mass deficit, in 7/11. There were no statistical differences between the subgroups. Conclusions: In patients with glycogenosis type I, there was deficit in growth and muscle mass, but no differences were found between the subgroups (Ia and Ib). Although the diet did not exceed the adequacy of carbohydrates, about 1/3 of the patients presented obesity, probably due to higher energy intake.

show abstract

Section: Discussioncontrasting

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Anthropometric and Dietary Assessment of Patients With Glycogenosis Type I

Jorge

Tommaso

Hessel

2021

Rev. paul. pediatr.

View full text Add to dashboard Cite

show abstract

“…This can only be achieved by a stringent dietary regime with frequent daytime meals and continuous night‐time gastrostomy feeds. Corn starch is given frequently during the daytime to promote euglycaemia, and a glucose polymer preparation or a formula free of sucrose and lactose are used continuously at night (4). The daily total energy supply should consist of 60–65% carbohydrates, 10–15% protein and 20–30% fat.…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic critical hypoglycaemia: a dangerous presentation of glycogen storage disease type Ib in infancy

2011

View full text Add to dashboard Cite

show abstract

“…TC had decreased from 6.18 ± 2.47 mmol/L to 5.61 ± 1.84 mmol/L(P = 0.02) and TG had decreased from 11.17 ± 9.85 mmol/L to 6.81 ± 5.97 mmol/L(P = 0.01). In another study on 19 GSDI patients treated with nocturnal gastric tube infusion since they were one year old, lipid level decreased signi cantly but did not return to normal level [18].…”

Section: Lipid Levels In Gsdia Patient After Raw Cornstarch Treatment Tmentioning

confidence: 96%

Lipid Status and Linear Relationship Between Total Cholesterol and Triglycerides in Glycogen Storage Disease Type Ia

Zhang

Yuan

et al. 2020

Preprint

View full text Add to dashboard Cite

Background Glycogen storage disease type Ia(GSDIa) is a glucose metabolic disorder disease. GSDIa patients are characterized by hypoglycemia, hepatomegaly, hyperlipidemia, and hyperlactacidemia. This study aims to review the lipid status, explore lipid treatment targets, and assess preferable lipid-lowering drugs. Methods Clinical data on GSDIa patients’ characteristics were collected. Diet control and raw cornstarch treatment were used to maintain normal blood glucose and lipid level. Some patients were given lipid-lowering drugs. We compared the lipid levels before and after each treatment. Results A total of 163 GSDIa patients were enrolled in this study. The lipid level was assessed 773 times. After treatment with raw cornstarch, TG level has significantly decreased by 30 ± 50%(P < 0.001). There was no change in TC level. Thirty patients regularly took atorvastatin or fibrates for more than one year. The therapeutic effect of atorvastatin was better than fibrates. The total cholesterol(TC) was positively correlated with TG after treatment, resulting in the following regression equation: TG = 1.63*TC-2.86. Conclusion Patients with GSDIa have significant abnormalities in blood lipid metabolism. We found that there was a linear correlation between TC and TG, which was TG = 1.63*TC-2.86. Because the complications of hyperlipidemia are caused mainly by TC, thereby, as long as the TC remains normal, we could set a TG target to allow a certain degree of hypertriglyceridemia. The bound of Chinese Children's TC normal level (5.18 mmol/L) was used in the established regression equation to obtain TG = 5.58 mmol/L. We, therefore, targeted to keep TG level below 5.58 mmol/L. This study found that the therapeutic effect of atorvastatin was better than fibrates.

show abstract

Type I glycogen storage disease: favourable outcome on a strict management regimen avoiding increased lactate production during childhood and adolescence

Abstract: avoiding increased lactate production by keeping the blood glucose concentration permanently in the "high normal range" seems to be crucial for growth according to the genetic potential.

Cited by 14 publications

References 16 publications

Anthropometric and Dietary Assessment of Patients With Glycogenosis Type I

Anthropometric and Dietary Assessment of Patients With Glycogenosis Type I

Asymptomatic critical hypoglycaemia: a dangerous presentation of glycogen storage disease type Ib in infancy

Lipid Status and Linear Relationship Between Total Cholesterol and Triglycerides in Glycogen Storage Disease Type Ia

Contact Info

Product

Resources

About