A pilot-study of body composition in GSD I and III managed with nocturnal UCCS.

Wartena, S.C (2014) A pilot-study of body composition in GSD I and III managed with nocturnal UCCS. thesis, Medicine.

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Abstract

Glycogen storage diseases (GSD) type I and III are rare inherited metabolic diseases affecting the enzymes in the metabolic pathway of glycogen synthesis and breakdown. Since the (nocturnal) fasting intolerance is managed successfully with exogenous carbohydrates, such as uncooked cornstarch (UCCS) or continuous nocturnal gastric drip feeding (CNGDF), longterm complications have emerged and research focus has shifted. Researchers have shared their concerns about under- and overtreatment and there effects on the long-term. UCCS management raises the blood glucoses more than CNGDF, what could induce glycogen and fat storage. The study, a case-control study, with healthy controls was conducted at the genetic service of Genetics Department –Hospital de Clínicas de Porto Alegre (HCPA/UFRGS), Porto Alegre (RS) - Brazil. Anthropometry and body composition were measured. To measure the body composition a bioelectrical impedance analyzer (BIA) was used. Dietary intake from patients was retrieved by 24-hour diet recall. The prediction of average energy requirements (EAR) was done by calculating the basal metabolic rate in Mega joules per day with the Oxford prediction. Lactate and triglyceride levels were used as indication of metabolic control for GSD Ia and Ib. We used descriptive statistics to interpret the data. Generally GSD patients were shorter and heavier, what resulted in a higher BMI. When BMI classification was used the prevalence of overweight and obese participants was higher in the group of GSD patients, 71.4% versus 42.9% in the group of controls. The higher prevalence of the BMI classifications overweight and obesity was supported by a higher median percentage of fat mass in GSD patients (24.2%) compared to the controls (17.0%). However when patient cases were matched with healthy controls, only 4 out of 7 patients showed a higher fat percentage in comparison to their controls. Possible variables that contributed to this differences were analyzed: type of GSD, metabolic control, percentage of UCCS of the total caloric intake, percentage of total caloric intake of the EAR and percentage of carbohydrates in the total diet. No clear link could be discovered between the variables and the fat percentage. In summary we concluded that patients managed with nocturnal UCCS, do not have a significant higher fat percentage in comparison the healthy controls. Furthermore we could not identify an association between other variables that could affect the fat percentage. This could indicate that GSD patients do not have a higher risk of diseases associated with a higher fat percentage. These findings are contradicting previous reports and presumptions.

Item Type:	Thesis (Thesis)
Supervisor name:	Derks, T.G.J. and Kallenberg, C.G.M.
Supervisor name:	Schwartz, I. V. D. and Genetics Department and Hospital de Clínicas de Porto Alegre and Porto Alegre (RS) - Brazil
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 11:02
Last Modified:	25 Jun 2020 11:02
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/2182

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