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2. Disorders of Creatine Metabolism

Author

Stöckler-Ipsiroglu, Sylvia, Braissant, Olivier, Schulze, Andreas, Blau, Nenad, editor, Dionisi Vici, Carlo, editor, Ferreira, Carlos R., editor, Vianey-Saban, Christine, editor, and van Karnebeek, Clara D. M., editor

Published
2022
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3. Creatine Deficiency Syndromes

Author

Stöckler-Ipsiroglu, Sylvia, Mercimek-Mahmutoglu, Saadet, Salomons, Gajja S., Saudubray, Jean-Marie, editor, van den Berghe, Georges, editor, and Walter, John H., editor

Published
2012
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4. Disorders of Creatine Metabolism

Author

Stöckler-Ipsiroglu, Sylvia, Battini, Roberta, DeGrauw, Ton, Schulze, Andreas, Blau, Nenad, editor, Leonard, James, editor, Hoffmann, Georg F., editor, and Clarke, Joe T. R., editor

Published
2006
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5. Creatine Deficiency Syndromes

Author

Stöckler-Ipsiroglu, Sylvia, Salomons, Gajja S., Fernandes, John, editor, Saudubray, Jean-Marie, editor, van den Berghe, Georges, editor, and Walter, John H., editor

Published
2006
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6. Disorders of Creatine Metabolism

Author

Stöckler-Ipsiroglu, Sylvia, Stromberger, Carmen, Item, Chike B., Mühl, Adolf, Blau, Nenad, editor, Duran, Marinus, editor, Blaskovics, Milan E., editor, and Gibson, K. Michael, editor

Published
2003
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17. 89. Progression of organ manifestations upon enzyme replacement therapy in a patient with mucopolysaccharidosis type IH prior to hematopoietic stem cell transplantation

Author

Mercimek-Mahmutoglu, Saadet, primary, Lillquist, Yolanda, additional, Davis, Jeff, additional, Reilly, Christopher, additional, Human, Derek, additional, Waters, Paula J., additional, Paschke, Eduard, additional, Clarke, Lorne A., additional, and Stoeckler-Ipsiroglu, Sylvia, additional

Published
2009
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20. Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene

Author

Plecko, Barbara, primary, Paul, Karl, additional, Paschke, Eduard, additional, Stoeckler-Ipsiroglu, Sylvia, additional, Struys, Eduard, additional, Jakobs, Cornelis, additional, Hartmann, Hans, additional, Luecke, Thomas, additional, di Capua, Matteo, additional, Korenke, Christoph, additional, Hikel, Christiane, additional, Reutershahn, Elke, additional, Freilinger, Michael, additional, Baumeister, Fritz, additional, Bosch, Friedrich, additional, and Erwa, Wolfgang, additional

Published
2006
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21. Oral β-Hydroxybutyrate Supplementation in Two Patients with Hyperinsulinemic Hypoglycemia: Monitoring of β-Hydroxybutyrate Levels in Blood and Cerebrospinal Fluid, and in the Brain by In Vivo Magnetic Resonance Spectroscopy

Author

Plecko, Barbara, primary, Stoeckler-Ipsiroglu, Sylvia, additional, Schober, Edith, additional, Harrer, Georg, additional, Mlynarik, Vladimir, additional, Gruber, Stephan, additional, Moser, Ewald, additional, Moeslinger, Dorothea, additional, Silgoner, Heinz, additional, and Ipsiroglu, Osman, additional

Published
2002
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23. Oral -Hydroxybutyrate Supplementation in Two Patients with Hyperinsulinemic Hypoglycemia Monitoring of -Hydroxybutyrate Levels in Blood and Cerebrospinal Fluid, and in the Brain by In Vivo Magnetic Resonance Spectroscopy

Author

PLECKO, BARBARA, STOECKLER-IPSIROGLU, SYLVIA, SCHOBER, EDITH, HARRER, GEORG, MLYNARIK, VLADIMIR, GRUBER, STEPHAN, MOSER, EWALD, MOESLINGER, DOROTHEA, SILGONER, HEINZ, AND, and IPSIROGLU, OSMAN

Abstract

In persistent hyperinsulinemic hypoglycemia of infancy, ketone body concentrations are abnormally low at times of hypoglycemia, depriving the brain of its most important alternative fuel. The neuroprotective effect of endogenous ketone bodies is evidenced by animal and human studies, but knowledge about exogenous supply is limited. Assuming that exogenous ketone body compounds as a dietetic food might replace this alternative energy source for the brain, we have monitored the fate of orally supplemented dl sodium -hydroxybutyrate (-OHB) in two 6-mo-old infants with persistent hyperinsulinemic hypoglycemia for 5 and 7 mo, while on frequent tube-feedings and treatment with octreotide. Near total (95) pancreatectomy had been ineffective in one patient and was refused in the other. In blood, concentrations of -OHB increased to levels comparable to a 16- to 24-h fast while on dl sodium -OHB 880 to 1000 mg/kg per day. In cerebrospinal fluid, concentrations of -OHB increased to levels comparable to a 24- to 40-h fast, after single dosages of 4 and 8 g, respectively. High ratios of -OHB to acetoacetate indicated exogenous origin of -OHB. An increase of intracerebral concentrations of -OHB could be demonstrated by repetitive single-voxel proton magnetic resonance spectroscopy by a clear doublet at 1.25 ppm. Oral dl sodium -OHB was tolerated without side effects. This first report on oral supplementation of dl sodium -OHB in two patients with persistent hyperinsulinemic hypoglycemia demonstrates effective uptake across the blood–brain barrier and could provide the basis for further evaluation of the neuroprotective effect of -OHB in conditions with hypoketotic hypoglycemia.

Published
2002

25. Ziliendiagnostik

Author

Nüßlein, Thomas, Griese, M., Nicolai, T., Koletzko, B., editor, Reinhardt, D., editor, and Stöckler-Ipsiroglu, Sylvia, editor

Published
2002
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39. Thrombosen im Kindesalter

Author

Kosch, Andrea, von Kries, Rüdiger, Nowak-Göttl, Ulrike, Koletzko, B., editor, Reinhardt, D., editor, and Stöckler-Ipsiroglu, Sylvia, editor

Published
2002
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41. Meteorismus

Author

Behrens, R., Koletzko, B., editor, Reinhardt, D., editor, and Stöckler-Ipsiroglu, Sylvia, editor

Published
2002
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44. Long-term follow-up of patients with congenital hyperinsulinism in Austria.

Author

Mercimek-Mahmutoglu S, Rami B, Feucht M, Herle M, Rittinger O, Stoeckler-Ipsiroglu S, and Schober E

Subjects
Adolescent, Adult, Austria, Child, Child, Preschool, Congenital Hyperinsulinism complications, Female, Follow-Up Studies, Humans, Infant, Newborn, Intellectual Disability etiology, Male, Pancreatectomy, Retrospective Studies, Time Factors, Congenital Hyperinsulinism surgery
Abstract

Aim: To assess the neurological and clinical long-term outcome of patients diagnosed with congenital hyperinsulinism (CHI) in Austria., Patients and Methods: Fourteen patients diagnosed with CHI (1978-2000) were investigated retrospectively by reviewing hospital records. Thirteen of them were evaluated with either a questionnaire or clinical, neurological and biochemical investigations (age at evaluation 4.2-25.5 years) in a follow-up study in the year 2004., Results: Fifty percent of the patients needed a pancreatectomy. The prevalence of mental retardation was 31%, of epilepsy 15% and of pancreatic insufficiency 14%. None of our patients had developed diabetes mellitus. Additionally the prevalence of obesity was 43% in patients after pancreatectomy. Sixty-nine percent of the patients had no further treatment at the time of follow-up., Conclusion: Despite early diagnosis and intensive treatment, 31% of the patients presented with mental retardation.

Published
2008

46. Oral beta-hydroxybutyrate supplementation in two patients with hyperinsulinemic hypoglycemia: monitoring of beta-hydroxybutyrate levels in blood and cerebrospinal fluid, and in the brain by in vivo magnetic resonance spectroscopy.

Author

Plecko B, Stoeckler-Ipsiroglu S, Schober E, Harrer G, Mlynarik V, Gruber S, Moser E, Moeslinger D, Silgoner H, and Ipsiroglu O

Subjects
3-Hydroxybutyric Acid blood, 3-Hydroxybutyric Acid cerebrospinal fluid, Administration, Oral, Blood Glucose, Dietary Supplements, Electrocardiography, Electroencephalography, Fatty Acids, Nonesterified analysis, Fatty Acids, Nonesterified blood, Fatty Acids, Nonesterified cerebrospinal fluid, Humans, Infant, Newborn, Ketone Bodies analysis, Ketone Bodies blood, Ketone Bodies cerebrospinal fluid, Neuroprotective Agents administration & dosage, Recurrence, 3-Hydroxybutyric Acid administration & dosage, Brain Chemistry, Hyperinsulinism drug therapy, Hypoglycemia drug therapy, Magnetic Resonance Spectroscopy
Abstract

In persistent hyperinsulinemic hypoglycemia of infancy, ketone body concentrations are abnormally low at times of hypoglycemia, depriving the brain of its most important alternative fuel. The neuroprotective effect of endogenous ketone bodies is evidenced by animal and human studies, but knowledge about exogenous supply is limited. Assuming that exogenous ketone body compounds as a dietetic food might replace this alternative energy source for the brain, we have monitored the fate of orally supplemented DL sodium beta-hydroxybutyrate (beta-OHB) in two 6-mo-old infants with persistent hyperinsulinemic hypoglycemia for 5 and 7 mo, while on frequent tube-feedings and treatment with octreotide. Near total (95%) pancreatectomy had been ineffective in one patient and was refused in the other. In blood, concentrations of beta-OHB increased to levels comparable to a 16- to 24-h fast while on DL sodium beta-OHB 880 to 1000 mg/kg per day. In cerebrospinal fluid, concentrations of beta-OHB increased to levels comparable to a 24- to 40-h fast, after single dosages of 4 and 8 g, respectively. High ratios of beta-OHB to acetoacetate indicated exogenous origin of beta-OHB. An increase of intracerebral concentrations of beta-OHB could be demonstrated by repetitive single-voxel proton magnetic resonance spectroscopy by a clear doublet at 1.25 ppm. Oral DL sodium beta-OHB was tolerated without side effects. This first report on oral supplementation of DL sodium beta-OHB in two patients with persistent hyperinsulinemic hypoglycemia demonstrates effective uptake across the blood-brain barrier and could provide the basis for further evaluation of the neuroprotective effect of beta-OHB in conditions with hypoketotic hypoglycemia.

Published
2002
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