Safety issues associated with dietary management in patients with hepatic glycogen storage disease.
Mol Genet Metab
; 125(1-2): 79-85, 2018 09.
Article
in En
| MEDLINE
| ID: mdl-30037503
ABSTRACT
INTRODUCTION:
Hepatic glycogen storage diseases (GSDs) are a group of inherited disorders of carbohydrate metabolism for which dietary management is the cornerstone. Safety and acute complications associated with dietary management have been poorly documented. We hypothesized that safety issues and complications associated with dietary management are prevalent amongst patients with these ultra-rare disorders.METHODS:
A questionnaire was developed consisting of 40 questions and was distributed via eight GSD patient organizations from multiple countries. Respondents were (caregivers of) patients with self-reported hepatic GSD.RESULTS:
249 GSD patients from 26 countries responded with a median age of 14.8â¯years (range 0.5-66.1). Although management was considered safe by 71% of patients, 51% reported at least one acute complication associated with dietary management, with a total number of 425 reported complications. Most frequently reported causes were not waking up by an alarm clock (nâ¯=â¯70), forgetting a meal (nâ¯=â¯57) and infections (nâ¯=â¯43). Most frequently reported complications were hypoglycemia (nâ¯=â¯112), hospital admissions (nâ¯=â¯79) and drowsiness (nâ¯=â¯74). Most complications occurred before the age of 12â¯years (82%; 637/774 total number of reported events) and during night time (63%; 340/536). Only 61% (152/249) of the GSD patients reported using a written emergency protocol.CONCLUSIONS:
Safety issues and complications associated with dietary management are prevalently reported by (caregivers of) 249 GSD patients. A discrepancy has been observed between the patient's perspective on safety of dietary management and occurrence of complications as a result of dietary management.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Glycogen Storage Disease
/
Diet
/
Liver
Type of study:
Risk_factors_studies
Limits:
Adolescent
/
Adult
/
Aged
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
/
Middle aged
Language:
En
Journal:
Mol Genet Metab
Journal subject:
BIOLOGIA MOLECULAR
/
BIOQUIMICA
/
METABOLISMO
Year:
2018
Document type:
Article
Affiliation country: