Results 201 to 210 of about 25,377 (250)

Renal Disease in Type I Glycogen Storage Disease

New England Journal of Medicine, 1988
Although kidney enlargement occurs in Type I glycogen storage disease, renal disease has not been considered a major problem. Death from renal failure in three patients known to us prompted a study of renal function in this disorder. Of the 38 patients with Type I glycogen storage disease under our care, the 18 children under 10 years old had normal ...
Yuan-Tsong Chen, R A Coleman, Chen Yuan-Tsong   +2 more
exaly   +4 more sources

Guidelines for management of glycogen storage disease type I - European Study on Glycogen Storage Disease Type I (ESGSD I)

European Journal of Pediatrics, 2002
Life-expectancy in glycogen storage disease type I (GSD I) has improved considerably. Its relative rarity implies that no metabolic centre has experience of large series of patients and experience with long-term management and follow-up at each centre is limited. There is wide variation in methods of dietary and pharmacological treatment.
Philippe Labrune, Kurt Ullrich, Labrune Philippe   +2 more
exaly   +5 more sources

GLYCOGEN STORAGE DISEASE: TYPE I

Archives of Pediatrics & Adolescent Medicine, 1969
To the Editor .—I read with interest Dr. Richard Fine's article inThe Journal(Amer J Dis Child115:238-331 [March] 1968). I would like to make some comments with regard to the author's statement: "Retinal changes have not been previously described in patients with GSD Type I." Newer mentioned Von Gierke's disease among conditions in which lipemia ...
R N, Fine, W A, Wilson, G N, Donnell
openaire   +4 more sources

Menorrhagia in Patients With Type I Glycogen Storage Disease

Obstetrics & Gynecology, 2013
To evaluate menorrhagia in a cohort of women with glycogen storage disease type I because it appears to be an under-recognized problem in females of reproductive age.A retrospective chart review was performed on 13 menstruating patients with glycogen storage disease type I (age 23-48 years) for a diagnosis of menorrhagia.Nine (69%) (confidence interval
Stephanie L, Austin, Areeg H, El-Gharbawy, Vellore G, Kasturi, Andra, James, Priya S, Kishnani   +4 more
openaire   +2 more sources

Intestinal function in glycogen storage disease type I

Journal of Inherited Metabolic Disease, 2002
AbstractGlycogen storage disease type I (GSD I) (McKusick 232200) is caused by inherited defects of the glucose‐6‐phosphatase complex. Patients with GSD Ia as well as patients with GSD Ib may suffer from intermittent diarrhoea, which seems to worsen with age. The cause of this diarrhoea is unknown.
Visser, G, Rake, JP, Kokke, FTM, Nikkels, PGJ, Sauer, PJJ, Smit, GPA   +5 more
openaire   +3 more sources

Hepatocellular carcinoma in type I glycogen storage disease

Hepatology, 1988
Patients suffering from Type I glycogen storage disease frequently develop hepatic tumors. Some of these were classified as carcinoma, with the majority of tumors representing benign adenomata. However, no evidence exists of malignant transformation of adenomata in these patients.
J, Limmer, W E, Fleig, D, Leupold, R, Bittner, H, Ditschuneit, H G, Beger   +5 more
openaire   +2 more sources

Insulin Secretion in Type I Glycogen Storage Disease

Diabetes, 1969
Patients with type I glycogen storage disease (glucose-6- phosphatase deficiency) have marked hypoglycemia in infancy but tend to become normoglycemic as they grow older. In addition, these patients characteristically demonstrate abnormal glucose tolerance curves.
D H, Lockwood, T J, Merimee, P J, Edgar, M L, Greene, W Y, Fujimoto, J E, Seegmiller, R R, Howell   +6 more
openaire   +2 more sources

Pulmonary hypertension in type I glycogen storage disease

Pediatric Cardiology, 1990
Two cases of pulmonary hypertension associated with type I glycogen storage disease (type I GSD) are reported. Before the development of pulmonary hypertension, patient 1 had been treated with dietary therapy with nocturnal gastric-drip infusion and zyloric therapy.
K, Hamaoka, M, Nakagawa, N, Furukawa, T, Sawada   +3 more
openaire   +2 more sources

Intestinal absorption in type I glycogen storage disease

The Journal of Pediatrics, 1969
Although intermittent diarrhea in type I glycogen storage disease has not been emphasized previously, we have frequently observed it as a clinical manifestation in our patients. In an attempt to delineate the etiology of the diarrhea, 8 patients with type I glycogen storage disease were studied.
R N, Fine, M D, Kogut, G N, Donnell
openaire   +2 more sources