solated ACTH deficiency is a very uncommon cause of hypoadrenocorticism, with less than 200 cases reported in the literatures. The clinical presentation can be similar to that of primary adrenal insufficiency, but there is a greater tendency for hypoglycemia and absence of hyperpigmentation. The diagnosis is established by demonstrating hypocortisolism with undetectable serum levels of ACTH, normal adrenal responsiveness to prolonged ACTH infusion, and an absent ACTH response to insulin-induced hypoglycemia. Other endocrine function is normal.We experienced a case of isolated ACTH deficiency in 34 years old female who was admitted due to fever and drowsy mentality.So we present this case with a review of literatures.
Addison Disease ; Adrenocorticotropic Hormone ; Diagnosis ; Female ; Fever ; Humans ; Hyperpigmentation ; Hypoglycemia

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

Insulinoma is a rare tumor occurring more often in the older age range. Because of the neuroglycopenic symptoms initial misdiagnosis or the delayed diagnosis are frequent. We recently experienced a case of insulinoma in a 50-year-old female with the symptoms of complex partial seizure, who had been treated with the antiepileptic drugs. Hypoglycemia was detected and the parenteral supplement of glucose rapidly reversed her symptoms, and the prolonged fasting test showed the pattern of insulinoma. Curative surgery was performed. So we report a case of insulinoma with the review of literature.
Anticonvulsants ; Delayed Diagnosis ; Diagnostic Errors ; Fasting ; Female ; Glucose ; Humans ; Hypoglycemia ; Insulinoma* ; Middle Aged ; Seizures*

Focal nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Because it is difficult to localize and detect with current imaging modalities, nesidioblastosis is challenging for biliary-pancreatic surgeons. ⁶⁸Gallium-DOTA-D-Phe¹-Tyr³-octreotide PET scanning and ¹¹¹indium-pentetreotide diethylene triamine pentaacetic acid octreotide scanning may be superior to conventional imaging modalities in determining the localization of nesidioblastosis. We report the successful surgical treatment of a 54-year-old woman with focal hyperplasia of the islets of Langerhans, who experienced frequent hypoglycemic symptoms and underwent various diagnostic examinations with different results.
Adult ; Diagnosis* ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Islets of Langerhans ; Middle Aged ; Nesidioblastosis* ; Octreotide ; Positron-Emission Tomography* ; Surgeons

Focal nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Because it is difficult to localize and detect with current imaging modalities, nesidioblastosis is challenging for biliary-pancreatic surgeons. ⁶⁸Gallium-DOTA-D-Phe¹-Tyr³-octreotide PET scanning and ¹¹¹indium-pentetreotide diethylene triamine pentaacetic acid octreotide scanning may be superior to conventional imaging modalities in determining the localization of nesidioblastosis. We report the successful surgical treatment of a 54-year-old woman with focal hyperplasia of the islets of Langerhans, who experienced frequent hypoglycemic symptoms and underwent various diagnostic examinations with different results.
Adult ; Diagnosis* ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Islets of Langerhans ; Middle Aged ; Nesidioblastosis* ; Octreotide ; Positron-Emission Tomography* ; Surgeons

Although hypoglycemia may be common among neonates, brain injuries resulting from isolated neonatal hypoglycemia are rare. The condition may cause neurological symptoms such as stupor, jitteriness, and seizures, though in their absence, diagnosis is delayed or difficult. Hypoglycemia was diagnosed in a three-day-old neonate after he visited the emergency department with loose stool, poor oral intake, and decreased activity, first experienced two days earlier. Two days after his visity, several episodes of seizure occurred. T2 and diffusion-weighted magnetic resonance (MR) scanning, performed at 11 days of age, revealed bilateral and symmetrical high signal intensity lesions in occipital, parietal, and temporal lobes. We report the MR findings of hypoglycemic encephalopathy in a neonate.
Brain Injuries* ; Brain* ; Diagnosis ; Emergency Service, Hospital ; Humans ; Hypoglycemia* ; Infant, Newborn ; Rabeprazole ; Seizures ; Stupor ; Temporal Lobe

Insuliin secreting tumor is 70% prevalent disease in female and predoadnant in forth and sixth deeade. The incidence of insulinoma is one case per 250,000 patient-years. Insulinoma in pregnancy was extremely rare, and the prevalence was not reported. The diagnosis of an insulinoma is depend on demonstration of hypoglycemia with high insulin and C-peptide levels. Immunoreactive insulin/plasma glucose ratio0.3 in particular support the diagnosis of an insulinoma. Fetal complication would be developed because of hypoglycemia. In approximately half of the cases reported, surgical exploration was done during pregrancy, the remainder were treated after delivery. Insulinoma poses serious diagnostic and therapeutic problems when she is pregnant. We experienced a case of insulinoma in pregnancy that represented Whipples triad and was treated by surgical intervention.
C-Peptide ; Diagnosis ; Female ; Glucose ; Humans ; Hypoglycemia ; Incidence ; Insulin ; Insulinoma* ; Pregnancy* ; Prevalence

Congenital hyperinsulinism (CHI), the most important cause of hyperglycemia in early infancy, is a heterogenous disease characterized by dysregulation of insulin secretion. Mutations in five proteins have been associated with CHI: sulfonyl urea receptor 1; Kir 6.2; glucokinase; glutamate dehydrogenase and mitochondrial enzyme short-chain 3-hydroxyacyl-coenzyme A dehydrogenase. Early recognition of hypoglycemia, diagnosis of CHI and appropriate management of the hypoglycemia are of the utmost importance to prevent neurologic damage. We report a case of persistent hyperinsulinemic hypoglycemia in 8-month-old male infant. This patient has no mutation in previously mentioned genes. Treatment with diazoxide was successful without any severe side effects in this patient.
Congenital Hyperinsulinism* ; Diagnosis ; Diazoxide* ; Glucokinase ; Glutamate Dehydrogenase ; Humans ; Hyperglycemia ; Hyperinsulinism ; Hypoglycemia ; Infant ; Insulin ; Male ; Oxidoreductases ; Urea

Blood Glucose ; analysis ; Capillaries ; metabolism ; Humans ; Hypoglycemia ; diagnosis ; Infant, Newborn ; Neonatal Screening ; methods ; Reference Standards

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