Brain Injuries ; etiology ; Humans ; Hypoglycemia ; complications ; Infant, Newborn

A boy aged 11 years was admitted due to intermittent weakness and difficulty in walking for 6 years, and hepatomegaly, glycopenia and unconsciousness for 4 years. The laboratory examinations showed severe metabolic acidosis, hypoglycemia, and abnormal liver function. CT scan showed marked liver enlargement with fat density shadow. The boy was given fluid infusion, correction of acidosis, intravenous injection of glucose, L-carnitine, compound vitamin B, and coenzyme Q10, but he was in a persistent coma and it was difficult to correct refractory metabolic acidosis and hypoglycemia. The boy died. Blood and urinary organic acid screening and gene detection confirmed that the boy had late-onset glutaric aciduria type II (GAIIc) caused by electron-transferring-flavoprotein dehydrogenase (ETFDH) gene defect. GAIIc is an inherited metabolic disease with a low incidence, resulting in a high misdiagnosis rate. GAIIc should be considered for children with recurrent weakness or reduced activity endurance, hypoglycemia, and marked liver enlargement with abnormal liver function. Urinary organic acid analysis and blood tandem mass spectrometry can help with the early diagnosis of GAIIc, and ETFDH gene analysis helps to make a confirmed diagnosis.
Child ; Hepatomegaly ; etiology ; Humans ; Hypoglycemia ; etiology ; Male ; Multiple Acyl Coenzyme A Dehydrogenase Deficiency ; diagnosis ; Muscle Weakness ; etiology

Hyperglycemia can result in severe adverse effects on the body. The mortality and morbidity of surgery are increased significantly in diabetic patients. The surgical stress-related hyperglycemia and insulin resistance can also produce the same adverse consequences. The metabolic state of the surgical patients, anesthesia method, glucose infusion, stress-induced neuroendocrine responses and insulin resistance can affect the perioperative blood glucose levels, resulting in poor clinical outcomes. The relationship between tight glycemic control and reducing post-operative mortality and morbidity is not clear. It's necessary to control blood sugar level during the perioperative period but the ideal state of glycemic control still needs a mult-center clinical trial evidence. It is generally believed that perioperative blood glucose level should be controlled below 10 mmol/L. The efficacy and safety of tight glycemic control needs further study.
Blood Glucose ; metabolism ; Humans ; Hyperglycemia ; etiology ; therapy ; Hypoglycemia ; prevention & control ; Perioperative Care

Blood Glucose ; analysis ; Humans ; Hypoglycemia ; diagnosis ; etiology ; pathology ; Male ; Somatostatinoma ; complications ; diagnosis ; pathology ; Young Adult

A 72-year-old man who had suffered several episodes of syncope was diagnosed as having hyperinsulinemic hypoglycemia. Although imaging studies and percutaneous transhepatic portal venous sampling did not reveal the existence of any tumors in the pancreas, distal pancreatectomy was performed because the possibility of a small pancreatic endocrine tumor could not be completely rejected. External examination of the surgically removed pancreas did not reveal any tumors. Microscopically, the pancreas exhibited diffuse islet cell hyperplasia without nesidioblastosis. The patient remains euglycemic and has tolerated 24-hour fasting without any medication for a period of 10 months after the operation.
Aged ; Case Report ; Human ; Hyperinsulinemia/etiology* ; Hyperplasia ; Hypoglycemia/etiology* ; Islets of Langerhans/surgery ; Islets of Langerhans/pathology* ; Male ; Pancreatectomy/methods ; Treatment Outcome

The medical records of six cases of nesidioblastosis were examined to determine the diagnostic approach, treatment, and neurologic sequelae. All six patients were male, and their ages at the onset of the disease ranged from one day to six months (mean 3.36 +/- 2.5 mo.). Initial clinical features were seizure, cyanosis, poor feeding, and apnea. Other subsequent symptoms were developmental delay, hyperactivity, and cold sweating. The Birth weight of the neonatal onset group was heavier than the postneonatal onset group (4.4 +/- 0.3 vs 3.26 +/- 0.04 kg). Before the diagnosis of hyperinsulinism, steroids of ACTH proved effective for seizure control. Initially, hyperinsulinemia (serum insulin greater than 10 microU/ml) was detected in four cases, but another two cases also showed hyperinsulinism by insulin/glucose(I/G) ratio greater than 0.3 during the fasting test. The glucagon response performed in 2 cases, showed normal and partial responses. Euglycemia was obtained by near total pancreatectomy (95% pancreatic resection)without malabsorption or persistent diabetes. In one case, nesidioblastoma coexisted with nesidioblastosis. Developmental delay was noted in three cases. In this group, the mean duration between symptom onset and operation was longer than the group without developmental delay (1.25 +/- 0.47 vs 0.38 +/- 0.19 yr).
Brain Damage, Chronic/*etiology ; Child, Preschool ; Developmental Disabilities/etiology ; Humans ; Hypoglycemia/blood/*etiology ; Infant ; Infant, Newborn ; Insulin/*blood ; Male ; Pancreatic Diseases/complications/*diagnosis/therapy ; Postoperative Complications/epidemiology

OBJECTIVEAbnormal glucose metabolism during pregnancy can result in significant adverse outcomes for newborns. This study was designed to investigate the effect of different degrees of impaired glucose tolerance on newborns.

METHODSGlucose challenge test (GCT) and oral glucose tolerance test (OGTT) were performed in pregnant women at 24-28 weeks of gestation. Based on the results of the two tests, their newborn infants were classified into five groups: gestational diabetes mellitus (GDM, n=182), gestational impaired oral glucose tolerance [GIGT, subdividing into GIGT 1 h (n=57) and GIGT 2-3 h groups (n=156)based on the occurrence time of abnormal blood glucose after glucose load], GCT abnormal but OGTT normal (only GCT abnormal, n=38) and normal glucose tolerance (control, n=1 025). The perinatal outcomes were compared among the five groups.

RESULTSThe incidences of macrosomia, large for gestational age (LGA), small for gestational age (SGA), neonatal hypoglycemia and premature birth in the GIGT (1 h) group were significantly higher than those in the control group, but similar to those in the GDM group. The incidences of macrosomia, SGA, neonatal hypoglycemia and premature birth in the GIGT (2-3 h) and the only GCT abnormal groups significantly decreased as compared with those in the GDM group, and similar to those in the control group. GIGT (1 h ) group showed higher incidence of neonatal hypoglycemia and premature birth than the GIGT (2-3 h) and the only GCT abnormal groups.

CONCLUSIONSDifferent degrees of maternal impaired glucose tolerance have different effects on newborns. As maternal GDM, maternal GIGT (1 h) may increase the risk of macrosomia, LGA, SGA, neonatal hypoglycemia and premature birth.

Diabetes, Gestational ; metabolism ; Female ; Fetal Macrosomia ; etiology ; Glucose ; metabolism ; Glucose Intolerance ; complications ; Glucose Tolerance Test ; Humans ; Hypoglycemia ; etiology ; Infant, Newborn ; Male ; Pregnancy ; Premature Birth ; etiology ; Retrospective Studies

BACKGROUND: Associations of acute glycemic complications with season and ambient temperature have been reported in general population with diabetes. However, little is known about the risks of acute glycemic complications in relation to season and ambient temperature in pregnant women, who are likely to be even more vulnerable. This work aimed to investigate the associations of season and ambient temperature with pregnancies complicated with hyperglycemia emergency or severe hypoglycemia. METHODS: Two separate case-control studies were nested within 150,153 pregnancies by women with type 1, type 2, or gestational diabetes between 2009 and 2014 in Taiwan. Hyperglycemia emergency (mainly diabetic ketoacidosis and hyperosmolar hyperglycemic state) and severe hypoglycemia occurred in 77 and 153 diabetic pregnancies (cases), respectively. Ten control pregnancies were randomly selected for each case by matching each case pregnancy on type of diabetes (i.e., T1DM, T2DM, or GDM), maternal age on the date of acute glycemic complication occurrence (i.e., index date), and "length of gestation at risk" (i.e., period between conception and index date). Meteorological parameters were retrieved from 542 meteorological monitoring stations across Taiwan during 2008-2014. Conditional logistic regression analysis with generalized estimation equation was separately performed to estimate the covariate adjusted odds ratios (ORs) of each of the two acute glycemic complications in association with season and ambient temperature within 30 days prior to the index date. RESULTS: Compared to summer, winter season was associated with a significantly elevated risk of severe hypoglycemia with an OR of 1.74 (95% confidence interval (CI) 1.08-2.79). The OR of hyperglycemic emergency was also elevated in winter season at OR of 1.88, but the significance is only marginal (95% CI 0.97-3.64, p = 0.0598). Subgroup analyses further noted that such seasonal variation was also observed in pregnancies with pre-pregnancy type 1 diabetes and gestational diabetes. On the other hand, ambient temperature was not significantly associated with the two acute glycemic complications. CONCLUSIONS A moderately but significantly elevated risk of severe hypoglycemia was found in pregnant women with diabetes during winter season, and such increased risk was more evident in pregnancies with T1DM.
Case-Control Studies ; Diabetes Mellitus, Type 1/complications* ; Female ; Humans ; Hypoglycemia/etiology* ; Incidence ; Pregnancy ; Pregnant Women ; Taiwan/epidemiology* ; Temperature

Prolonged administration of anterior hypophyseal, adrenocortical, or thyroid hormones is known to cause degeneration, degranulation and necrosis of the beta-cells in the Langerhans islets of the pancreas. However, the effects of extirpation of these endocrine glands upon the Langerhans islets has not been reported, a1though it is known that removal of any of these glands bring about hypoglycemia, decreased tissue uptake of glucose, and increased tissue sensitivity to insulin. The present investigation is studies of the morphologic alterations of the beta-cells in the Langerhans islets following hypophysectomy, adrenalectomy, or thyroidectomy in rats. Hypophysectomy, adrenalectomy, and thyroidectomy, all induce similar morphologic alterations in the beta-cells of the islets. These consist of increased beta-cell population, the accumlnation of beta-granules, and atrophy of the individual betacell. Therefore, these changes are considered to be not specific following the withdrawal of specific hormones but a common effect of the hypoglycemia due to removal of the hypophysis, adrenals, or thyroid glands. A similar common degeneration of the beta-cells due to hyperglycemia occurs when hormones of these endocrine glands are given excessively.
Adrenal Cortex Hormones/physiology ; Adrenalectomy* ; Animal ; Atrophy/etiology ; Blood Glucose ; Diabetes Mellitus/etiology ; Glycogen/metabolism ; Hyperglycemia/etiology ; Hypoglycemia/etiology ; Hypophysectomy* ; Insulin/secretion ; Islets of Langerhans/pathology* ; Liver Glycogen/metabolism ; Muscles/metabolism ; Myocardium/metabolism ; Necrosis/etiology ; Rats ; Staining and Labeling ; Thyroidectomy* ; Thyroxine/physiology

OBJECTIVETo analyze the etiology, clinical and laboratory characteristics of hepatic failure in 105 children.

METHODSThe clinical data of 105 children with hepatic failure treated in our hospital from January 1986 to June 2003 were retrospectively analyzed by EXCELL 2000 and t test.

RESULTS(1)Of the 105 children with hepatic failure, 9 were cases with fulminant hepatic failure, 38 with subacute hepatic failure and 58 with chronic hepatic failure. (2)Morbidity was the highest in 7-12 years old children (43/105, 41.0%) followed by infants (30/105, 28.6%). (3)CMV infection could be confirmed in 9 infants (30.0%), etiological diagnosis was not possible in 13 infants (43.3%). Etiological diagnosis could be confirmed in children over 1 year of age, which included hepatitis B (n=22, 29.3%), Wilson's disease (n=15, 20.0%), hepatitis A (n=10,13.3%). Etiology in 21 cases (28.0%) could not be confirmed. (4)Seventy-one cases (67.6%) had ascites, 34 of them (47.9%) had spontaneous peritonitis. Thirty-five cases were complicated with other infections. The commonest complication was pulmonary infection and sepsis was the next. Fifty-one cases (48.6%) had hydroelectrolyte imbalance. Forty-eight cases (46.2%) had hepatic encephalopathy, which may be subclinical in children under three years of age. (5)The incidence of hypoglycemia was 77.2%(71/92).

CONCLUSIONThe etiology of liver failure was related to age. CMV infection was the commonest in infants. HBV, HAV infection was the commonest in children over 1 year of age and Wilson?s disease was the next. It is necessary to prevent and manage the associated complications as early as possible such as spontaneous peritonitis, hepatic encephalopathy, hydroelectrolyte imbalance and hypoglycemia etc.

Adolescent ; Age Factors ; Child ; Child, Preschool ; Cytomegalovirus Infections ; complications ; Female ; Hepatic Encephalopathy ; etiology ; Hepatitis A ; complications ; Hepatitis B ; complications ; Hepatolenticular Degeneration ; complications ; Humans ; Hypoglycemia ; complications ; Infant ; Liver Failure ; etiology ; Liver Failure, Acute ; etiology ; Male ; Peritonitis ; complications