Trial reportJournal of inherited metabolic disease1997
Metabolic control and renal dysfunction in type I glycogen storage disease.
Trial report in Journal of inherited metabolic disease, 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
18 citing papers in PubMed, 52 citations in OpenAlex.
- Short-term effect of captopril on microalbuminuria in children with glycogen storage disease type Ia.Journal of inherited metabolic disease · 2000Trial
- Glycogen storage disease type Ia with a 17-year history of renal involvement: a case report.Journal of medical case reports · 2026Article
- The kidney in genetic metabolic disorders.Medizinische Genetik : Mitteilungsblatt des Berufsverbandes Medizinische Genetik e.V · 2026Article
- Integral kidney function assessment in pediatric patients with glycogen storage diseases.Frontiers in pediatrics · 2025Article
- Preserved renal function during long-term follow-up in children with chronic liver disease.Acta paediatrica (Oslo, Norway : 1992) · 2022Article
- Clinical features of gout in adult patients with type Ia glycogen storage disease: a single-centre retrospective study and a review of literature.Arthritis research & therapy · 2022Review
- Modifiable factors affecting renal preservation in type I glycogen storage disease after liver transplantation: a single-center propensity-match cohort study.Orphanet journal of rare diseases · 2021Article
- A preliminary study of telemedicine for patients with hepatic glycogen storage disease and their healthcare providers: from bedside to home site monitoring.Journal of inherited metabolic disease · 2018Article
- Tight metabolic control plus ACE inhibitor therapy improves GSD I nephropathy.Journal of inherited metabolic disease · 2017Article
- Glycogen Storage Disease type 1a - a secondary cause for hyperlipidemia: report of five cases.Journal of diabetes and metabolic disorders · 2013Article
- In search of proof-of-concept: gene therapy for glycogen storage disease type Ia.Journal of inherited metabolic disease · 2012Article
- Rescue administration of a helper-dependent adenovirus vector with long-term efficacy in dogs with glycogen storage disease type Ia.Gene therapy · 2012Article
- Hepatorenal correction in murine glycogen storage disease type I with a double-stranded adeno-associated virus vector.Molecular therapy : the journal of the American Society of Gene Therapy · 2011Article
- Glycogen storage diseases.Reviews in endocrine & metabolic disorders · 2003Review
- Delivery of glucose-6-phosphatase in a canine model for glycogen storage disease, type Ia, with adeno-associated virus (AAV) vectors.Gene therapy · 2002Article
- Glycogen storage disease type Ia: frequency and clinical course in Turkish children.Indian journal of pediatrics · 2000Article
- Liver transplantation for glycogen storage disease types I, III, and IV.European journal of pediatrics · 1999Review
- Glycogen storage disease type 1a in three siblings with the G270V mutation.Journal of inherited metabolic disease · 1999Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
This study was undertaken to determine the effect on renal function of continuous glucose therapy from early childhood. Twenty-three subjects, median age 13.9 years, range 5.9-26.9 years, with type I glycogen storage disease (GSDI) treated with continuous glucose therapy from a median age of 1.3 years, range 0.1-12.9 years, had 24 h monitoring of metabolites and glucoregulatory hormones on their home feeding regimen to assess metabolic control at approximately yearly intervals for a median duration of 8 years. During the most recent evaluation, 24 h urinary albumin excretion rate (AER), kidney size, and creatinine clearance (Ccr) were measured. CCr was unrelated to age and was increased (> 2.33 ml/s per 1.73 m2) in 10/23 (43%). Mean kidney length exceeded 2SD in 16/23 (70%). AER was normal in all five subjects < 10 years and was increased (> 10 micrograms/min) in 8/23 (35%), all > 10 years of age. AER was significantly greater in subject of similar age who started continuous glucose therapy later in childhood and was significantly higher in subjects with lower mean 24 h plasma glucose concentrations and higher mean 24 h blood lactate concentrations, both at the time of assessment of renal function and over the preceding 5 years. GSDI subjects with persistently elevated concentrations of blood lactate, serum lipids and uric acid are at increased risk of nephropathy. Optimal dietary therapy instituted early in life may delay, prevent, or slow the progression of renal disease.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.