glycogen and Infant--Newborn--Diseases

Topics: Adipose Tissue; Blood Glucose; Carbohydrate Metabolism; Female; Fetus; Glucose; Glycogen; Glycolysis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Insulin; Liver; Pregnancy; Pregnancy in Diabetics; Pyruvates

Topics: Congenital Abnormalities; Embryo, Mammalian; Female; Fetal Death; Fetal Diseases; Fetus; Glycogen; Humans; Hypoglycemia; Infant, Newborn; Infant, Newborn, Diseases; Insulin; Maternal-Fetal Exchange; Placenta Diseases; Pregnancy; Pregnancy in Diabetics

Topics: Catecholamines; Cyclic AMP; Endocrine System Diseases; Fructose-Bisphosphatase; Glucagon; Gluconeogenesis; Glucose-6-Phosphatase; Glycogen; Glycogen Synthase; Growth Hormone; Humans; Hydrocortisone; Hypoglycemia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Insulin; Ketosis; Liver; Metabolism, Inborn Errors; Phosphoenolpyruvate Carboxykinase (GTP); Pyruvate Carboxylase

Topics: Animals; Birth Weight; Blood Glucose; Child; Cortisone; Female; Gestational Age; Gluconeogenesis; Glucose; Glycogen; Homeostasis; Humans; Hypoglycemia; Infant Food; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Insulin; Insulin Antagonists; Insulin Secretion; Liver; Liver Glycogen; Maternal-Fetal Exchange; Pregnancy; Pregnancy Complications

Topics: Animals; Birth Weight; Blood Glucose; Carbohydrate Metabolism; Cardiomegaly; Female; Fetus; Glucose; Glycogen; Haplorhini; Humans; Hydrocortisone; Hypoglycemia; Infant, Newborn; Infant, Newborn, Diseases; Insulin; Lipids; Liver; Polycythemia; Pre-Eclampsia; Pregnancy; Prognosis; Rabbits; Sex Factors; Time Factors

Insulin secretion from pancreatic β-cells is impaired in all forms of diabetes. The resultant hyperglycaemia has deleterious effects on many tissues, including β-cells. Here we show that chronic hyperglycaemia impairs glucose metabolism and alters expression of metabolic genes in pancreatic islets. In a mouse model of human neonatal diabetes, hyperglycaemia results in marked glycogen accumulation, and increased apoptosis in β-cells. Sulphonylurea therapy rapidly normalizes blood glucose levels, dissipates glycogen stores, increases autophagy and restores β-cell metabolism. Insulin therapy has the same effect but with slower kinetics. Similar changes are observed in mice expressing an activating glucokinase mutation, in in vitro models of hyperglycaemia, and in islets from type-2 diabetic patients. Altered β-cell metabolism may underlie both the progressive impairment of insulin secretion and reduced β-cell mass in diabetes.

Topics: Animals; Apoptosis; Autophagy; Blood Glucose; Cell Line; Diabetes Mellitus, Type 2; Disease Models, Animal; Glucokinase; Glycogen; Humans; Hyperglycemia; Hypoglycemic Agents; In Vitro Techniques; Infant, Newborn; Infant, Newborn, Diseases; Insulin; Insulin-Secreting Cells; Mice; Mutation; Rats; Sulfonylurea Compounds

A girl with congenital limb weakness, mental retardation, and corneal ulceration died with respiratory insufficiency at age 4 years. Histochemistry of muscle biopsy showed only nonspecific myopathy, but electronmicroscopy revealed subsarcolemmal and intramyofibrillar accumulation of glycogen. Biochemical studies showed increased glycogen content of muscle with lack of phosphofructokinase. Phosphorylase b kinase activity was about 30% of normal. The relationship of the double enzyme deficiency to this unusual clinical picture is unclear.

Topics: Biopsy; Child, Preschool; Female; Glycogen; Glycogen Storage Disease; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Muscles; Muscular Diseases; Phosphofructokinase-1; Phosphorylase Kinase

The concurrence of the infantile and adult form of acid maltase deficiency in one family is presented. The muscle of both cases possesses residual activity. The glycogen amount in the infantile form is increased tremendously, while in the late-onset the amount of glycogen is normal.

Topics: alpha-Glucosidases; Female; Glucosidases; Glycogen; Glycogen Storage Disease; Glycogen Storage Disease Type II; Humans; Hydrogen-Ion Concentration; Infant, Newborn; Infant, Newborn, Diseases; Male; Middle Aged; Muscles

Chest X-rays of 24 hypoglycemic newborns were compared with those of a matched control group of newborns with normal blood glucose levels. In the hypoglycemic group heart size was found to be significantly greater than in the control group. No correlation could be established between the degree of cardiomegaly and the severity of hypoglycemia. 19 of the 24 hypoglycemic children were prematures or small for dates and it is postulated that the low glycogen stores in these infants do not meet the metabolic demands of the myocardium during its postpartum circulatory adaptation.

Topics: Cardiomegaly; Glycogen; Humans; Hypoglycemia; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Myocardium

Topics: Cell Nucleus; Cytoplasm; Endoplasmic Reticulum; Female; Glycogen; Humans; Hyperparathyroidism; Hyperplasia; Infant, Newborn; Infant, Newborn, Diseases; Microscopy, Electron; Parathyroid Glands

Topics: Delivery, Obstetric; Erythroblastosis, Fetal; Female; Fetus; Glucose; Glycogen; Humans; Hypoglycemia; Hypoxia; Infant, Newborn; Infant, Newborn, Diseases; Labor, Obstetric; Lipid Metabolism; Maternal-Fetal Exchange; Oxygen Consumption; Pregnancy; Pregnancy in Diabetics

Topics: Adenosine Triphosphate; Animals; Animals, Newborn; Brain; Brain Damage, Chronic; Cerebrosides; Cholesterol; DNA; Gangliosides; Glucose; Glycogen; Humans; Hypoglycemia; Infant, Newborn; Infant, Newborn, Diseases; Lipid Metabolism; Nerve Tissue Proteins; Organ Size; Phosphocreatine; Phospholipids; Rats; Sulfoglycosphingolipids

Topics: Animals; Basement Membrane; Blood Vessels; Glycogen; Histocytochemistry; Humans; Infant, Newborn; Infant, Newborn, Diseases; Male; Microscopy, Electron; Mitochondria; Muscles; Muscular Diseases; Myofibrils; Nerve Endings

Topics: Blood Platelets; Diabetes Mellitus; Glycogen; Glycogen Storage Disease; Histocytochemistry; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Leukemia; Leukemia, Lymphoid; Leukemia, Myeloid; Liver Cirrhosis; Microscopy, Phase-Contrast; Myeloproliferative Disorders; Polycythemia; Primary Myelofibrosis; Purpura, Thrombocytopenic; Remission, Spontaneous; Uremia; von Willebrand Diseases

Topics: Amino Acids; Animals; Animals, Newborn; Blood Glucose; Disease Models, Animal; Fasting; Fatty Acids, Nonesterified; Female; Gluconeogenesis; Glycogen; Glycolysis; Growth Hormone; Humans; Hypoglycemia; Infant, Newborn; Infant, Newborn, Diseases; Insulin; Ketones; Lipid Metabolism; Liver; Liver Glycogen; Pregnancy; Pregnancy in Diabetics; Rats; Swine

Topics: Biopsy; Endoplasmic Reticulum; Glycogen; Humans; Hyperbilirubinemia; Infant, Newborn; Infant, Newborn, Diseases; Liver; Microscopy, Electron; Phenylbutazone

Topics: Female; Fetal Diseases; Glucose; Glycogen; Growth Disorders; Growth Hormone; Humans; Hypoglycemia; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infant, Newborn, Diseases; Jaundice, Neonatal; Male; Placenta Diseases; Pre-Eclampsia; Pregnancy; Pregnancy Complications; Prognosis; Respiratory Distress Syndrome, Newborn

Topics: Female; Fetal Diseases; Glycogen; Histocytochemistry; Humans; Hypoxia; Infant, Newborn; Infant, Newborn, Diseases; Neutrophils; Pregnancy

Topics: Acne Vulgaris; Collagen; Cysts; Elastic Tissue; Erythema; Glycogen; Hair; Humans; Hydrogen-Ion Concentration; Infant, Newborn; Infant, Newborn, Diseases; Lymphatic System; Nails; Pigmentation; Pigmentation Disorders; Sebaceous Glands; Skin; Skin Diseases; Sweat Glands; Sweating

Topics: Female; Glycogen; Humans; Hypoglycemia; Infant, Newborn; Infant, Newborn, Diseases; Leucine; Lipid Metabolism; Maternal-Fetal Exchange; Pregnancy

Topics: Adolescent; Autopsy; Child; Child, Preschool; Female; Glycogen; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Liver; Male

Topics: Child; Diabetes Mellitus; Female; Glycogen; Heart Diseases; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Metabolic Diseases; Mothers; Pregnancy