Ketosis in Hepatic Glycogenosis

Overview

Journal Arch Dis Child

Specialty Pediatrics

Date 1972 Feb 1

PMID 4336232

Citations 11

Authors

J Fernandes

N A PIKAAR

Affiliations

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Citing Articles

Report of an Iranian child with chronic abdominal pain and constipation diagnosed as glycogen storage disease type IX: a case report.

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Clinical and genetic spectrum of GSD type 6 in Korea.

Hahn J, Lee H, Seong M, Kang G, Moon J, Ko J Orphanet J Rare Dis. 2023; 18(1):132.

PMID: 37264426 PMC: 10233917. DOI: 10.1186/s13023-023-02750-1.

Hepatic glycogen storage diseases: pathogenesis, clinical symptoms and therapeutic management.

Szymanska E, Jozwiak-Dziecielewska D, Gronek J, Niewczas M, Czarny W, Rokicki D Arch Med Sci. 2021; 17(2):304-313.

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Impaired Very-Low-Density Lipoprotein catabolism links hypoglycemia to hypertriglyceridemia in Glycogen Storage Disease type Ia.

Hoogerland J, Peeks F, Hijmans B, Wolters J, Kooijman S, Bos T J Inherit Metab Dis. 2021; 44(4):879-892.

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The use of sodium DL-3-Hydroxybutyrate in severe acute neuro-metabolic compromise in patients with inherited ketone body synthetic disorders.

Bhattacharya K, Matar W, Tolun A, Devanapalli B, Thompson S, Dalkeith T Orphanet J Rare Dis. 2020; 15(1):53.

PMID: 32070364 PMC: 7029565. DOI: 10.1186/s13023-020-1316-x.

References

Fernandes J, PIKAAR N . Hyperlipemia in children with liver glycogen disease. Am J Clin Nutr. 1969; 22(5):617-27. DOI: 10.1093/ajcn/22.5.617. View

SIDBURY Jr J, GITZELMANN R, Fisher J . The glycogenoses. Further observations on glycogen in erythrocytes of patients with glycogenosis. Helv Paediatr Acta. 1961; 16:506-16. View

LOWE C, Sokal J, Mosovich L, Sarcione E, Doray B . Studies in liver glycogen disease. Effects of glucagon and other agents on metabolic pattern and clinical status. Am J Med. 1962; 33:4-19. DOI: 10.1016/0002-9343(62)90272-3. View

KELSCH R, Oliver W . Studies on dietary correction of metabolic abnormalities in hepatorenal glycogenosis. Pediatr Res. 1969; 3(2):160-70. DOI: 10.1203/00006450-196903000-00008. View

Huijing F . AMYLO-1,6-GLUCOSIDASE ACTIVITY IN NORMAL LEUCOCYTES AND IN LEUCOCYTES OF PATIENTS WITH GLYCOGEN-STORAGE DISEASE. Clin Chim Acta. 1964; 9:269-72. DOI: 10.1016/0009-8981(64)90106-8. View

Matschke I, NEUBAUR J, Willms B, Wolf H . [Metabolism studies in children and young adults with glycogenoses]. Monatsschr Kinderheilkd (1902). 1969; 117(4):259-62. View

HAVEL R, Balasse E, Williams H, Kane J, SEGEL N . Splanchnic metabolism in von Gierke's disease (glycogenosis type I). Trans Assoc Am Physicians. 1969; 82:305-23. View

Sokal J, LOWE C, Sarcione E, Mosovich L, Doray B . STUDIES OF GLYCOGEN METABOLISM IN LIVER GLYCOGEN DISEASE (VON GIERKE'S DISEASE): SIX CASES WITH SIMILAR METABOLIC ABNORMALITIES AND RESPONSES TO GLUCAGON. J Clin Invest. 1961; 40(2):364-74. PMC: 290729. DOI: 10.1172/JCI104263. View

Fernandes J, VAN DE KAMER J . STUDIES ON THE UTILIZATION OF HEXOSES IN LIVER GLYCOGEN DISEASE. Pediatrics. 1965; 35:470-7. View

10.

NEUBAUR J, Willms B, SOLING H, Creutzfeldt W . [Gout as a complication of adult glycogen storage disease]. Arch Klin Med. 1969; 216(2):148-60. View

11.

PEDEN V . DETERMINATION OF INDIVIDUAL SERUM "KETONE BODIES", WITH NORMAL VALUES IN INFANTS AND CHILDREN. J Lab Clin Med. 1964; 63:332-43. View

12.

Howell R, ASHTON D, WYNGAARDEN J . Glucose-6-phosphatase deficiency glycogen storage disease. Studies on the interrelationships of carbohydrate, lipid, and purine abnormalities. Pediatrics. 1962; 29:553-65. View

13.

Rennert O, Mukhopadhyay D . Diazoxide in von Gierke's disease. Arch Dis Child. 1968; 43(229):358-61. PMC: 2019951. DOI: 10.1136/adc.43.229.358. View

14.

Huijing F, Fernandes J . X-chromosomal inheritance of liver glycogenosis with phosphorylase kinase deficiency. Am J Hum Genet. 1969; 21(3):275-84. PMC: 1706412. View

15.

VAN CREVELD , Huijing F . DIFFERENTIAL DIAGNOSIS OF THE TYPE OF GLYCOGEN DISEASE IN TWO ADULT PATIENTS WITH LONG HISTORY OF GLYCOGENOSIS. Metabolism. 1964; 13:191-4. DOI: 10.1016/0026-0495(64)90132-5. View

16.

TROUT D, ESTES Jr E, FRIEDBERG S . Titration of free fatty acids of plasma: a study of current methods and a new modification. J Lipid Res. 1960; 1:199-202. View

17.

HERS H . GLYCOGEN STORAGE DISEASE. Adv Metab Disord. 1964; 13:1-44. DOI: 10.1016/b978-1-4831-6748-0.50006-9. View

18.

Hug G, Schubert W . Serum insulin in type I glycogenosis. Effect of galactose or fructose administration. Diabetes. 1967; 16(11):791-5. DOI: 10.2337/diab.16.11.791. View