Diazoxide in von Gierke's disease.

Ketosis in Hepatic Glycogenosis

“…This is in disagreement with the findings of several authors (Howell et al, 1962;Hug and Schubert, 1967 Table II). Kelsch and Oliver, 1969;Rennert and Mukhopadhyay, 1968), though Field (1966) states that 'acetonuria and acetonaemia are inconsistently present', and 'several cases have been seen in which life-threatening acidosis supervened without the appearance of significant acetonaemia and acetonuria'.…”

“…Ketosis has generally been considered to be a characteristic feature of glycogen storage disease in childhood (van Creveld, , 1932Howell, Ashton, and Wyngaarden, 1962;Hug and Schubert, 1967; Kelsch and Oliver, 1969;Lowe et al, 1962;Rennert and Mukhopadhyay, 1968;Sidbury, Gitzelmann, and Fisher, 1961;Sokal et al, 1961).…”

“…*Present address: Sophia Children's Hospital and Neonatal Unit, Rotterdam Medical School, Rotterdam, The Netherlands. (Howell et al, 1962;Hug and Schubert, 1967;Kelsch and Oliver, 1969;Rennert and Mukhopadhyay, 1968). Because of the conflicting evidence we decided to gather more data on ketosis in patients with the three most common types of glycogenosis, e.g.…”

Glycogen storage disease, type 8.

“…Diazoxide therapy was initiated since it inhibits insulin release, increases adrenal medullary secretion of epinephrine and inhibits the cyclic AMP phosphodiesterase activity (Rennert and Mukhopadhyay, 1968); these should act in concert to increase intracellular cyclic AMP. In this patient fasting hypoglycaemia was ameliorated; however, insulin suppression did not occur.…”

Stability of Diazoxide in Extemporaneously Compounded Oral Suspensions

“…Diazoxide is used in the pediatric population since the 1960s to manage hypoglycemia [ 7 – 8 ], and von Gierke’s disease [ 9 ]. It is still considered the first line of treatment for hyperinsulinaemic hypoglycemia of infancy [ 10 – 11 ].…”