ABSTRACT
Objective To investigate the biochemical disturbances,nutritional assessment and nutritional interventions in hospitalized patients with glycogen storage disease (GSD) in a general hospital in Beijing and provide examples of advanced nutritional management for these patients.Methods We retrospectively reviewed 22 consecutive hospitalized patients with GSD diagnosed from January 1,2013 to January 1,2016 in Peking Union Medical College Hospital and analyzed their medical data,nutritional assessments,and interventions.Results These 22 GSD patients included 2 cases with GSD-Ⅰa,3 cases with GSD-Ⅰb,and 17 cases with GSD-Ⅲ.They were composed of 21 children (including a 6-month old infant and a 7-month old infant) and one 18-yearold man.Biochemical abnormalities:hypoglycemia (5/5),hyperlactacidemia (4/5),hyperlipidemia (4/5),hyperuricemia (4/5) were common in GSD-Ⅰ patients,while hypoglycemia (17/17),hyperlactacidemia (5/17),hyperlipidemia (7/17),and hyperuricemia (3/17) were also present among patients with GSD-Ⅲ.Growth and development assessments:among 5 patients with GSD-Ⅰ,the body weight was below the 10th percentile in of 4 patients and the height was below the 3rd percentile in 5 patients;among 17 patients with GSD-Ⅲ,the body weight was below the 10th percentile in 4 patients and the height was below the 10th percentile in 9 patients.Nutritional management condition:all the patients received nutritional assessment,dietary modulation and nutritional support.Twenty children or adolescents were prescribed with raw corn starch (RCS) every 4-6 hours,with a mean initial dose of (1.58±0.28) g and (1.21±0.33) g for patients with GSD-Ⅰ and GSD-Ⅲ,respectively;the other 2 infant aged 6-month old and 7-month old were regularly administered amino acid-based enteral nutritional agents.In addition,all the patients with GSD-Ⅲ were recommended high protein intake of 3 g/(kg · d) and received dietary guidance and modulation.Follow-up condition:5 cases with GSD-Ⅰ and 2 cases with GSD-Ⅲ stayed in track after discharge from our hospital.The mean follow-up duration was (480.40±246.16) d and (373.00± 108.89) d for patients with GSD-Ⅰ and GSD-Ⅲ,respectively.All the patients reported alleviated symptom of hypoglycemia,and the examinations also indicated improved fasting blood glucose,uric acid,lipid profile,and growth status.Conelusions Hypoglycemia,metabolic disturbances,and growth retardation are prevalent in both GSD-Ⅰ and GSD-Ⅲ patients.Potential biochemical abnormalities should not be ignored in GSD-Ⅲ patients,especially in young patients.Comprehensive nutrition assessment,regular administration of RCS,sufficient protein intake,and continuous patient follow-up and surveillance can help to alleviate symptoms,correct metabolic disturbances,and improve growth status of patients with GSD.