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

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

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

Nesidioblastosis is characterized by a diffuse proliferation of islet cells arising from pancreatic ducts and is the most common cause of hyperinsulinemic hypoglycemia in newborns and infantile. It is exceedingly rare in adults and no concensus regarding its diagnosis and management is available. We herein describe an elderly man with fasting hypoglycemia, inappropriate insulin hypersecretion. And pathologic examination of his pancreas revealed the characteristic finding of nesidioblastosis confirmed by immunohistochemical stain.
Adult ; Aged* ; Diagnosis ; Humans ; Hypoglycemia* ; Infant, Newborn ; Insulin ; Islets of Langerhans ; Nesidioblastosis* ; Pancreas ; Pancreatic Ducts

Pancreatic islet cell adenoma is a benign tumor of pancreatic beta-cell and a rare cause of hyperinsulinemic hypoglycemia in children. The authors experienced a case of pancreatic islet cell adenoma(Insulinoma)in a 11 year and 8 month old male who had frequent loss of consciousness and seizure. Enucleation was done after localization of tumor by selective celiac artery angiography and abdominal computed tomography. Diagnosis was confirmed by histologic findings as pancreatic-adenoma, gyriform growth pattern. A brief review of related literature was made.
Adenoma* ; Angiography ; Celiac Artery ; Child ; Diagnosis ; Humans ; Hyperinsulinism ; Hypoglycemia ; Infant ; Islets of Langerhans ; Male ; Seizures ; Unconsciousness

Nesidioblastosis is a rare disease characterized clinically by persistent hypoglycemia with inappropriately elevated circulating insulin concentration. Adequate early diagnosis should be established and subtotal pancreatectomy performed before itreversible cerebral damage caused by glucose deficit. The authors got a chance to anesthetize 56-day-old male patient for subtotal pancreatectomy because of nesidioblastosis, Following an induction of anesthesia with intravenous thiapental sodium 5 mg/kg and succinylchohne 1 mg/kg, endotracheal intubation was performed and anesthesia was maintained with pancuronium bromide and O2-N2O-enflurane. Intravenous fluid was maintained with 1-2-3 solution 30 ml and 15% D/W 40 ml mixed with 20 ml of 20 mEq/L NaCI.
Anesthesia* ; Early Diagnosis ; Glucose ; Humans ; Hypoglycemia ; Insulin ; Intubation, Intratracheal ; Male ; Nesidioblastosis* ; Pancreatectomy* ; Pancuronium ; Rare Diseases ; Sodium

The insulinoma is a rare tumor, usually small, solitary and benign with no prediction for any part of the pancreas. It is amendable to surgical cure but approximately 10percent are malignant and the peak incidence is encountered between ages 40~60. Surgery has been established as the treatment of choice. The reason for electing to operate rather than treat symptomatically is prevention of complications, such as increasing obesity, or prolonged and irreversible episodes of hypoglycemic coma with resultant permanent central nervous damage. During the anesthesia for a patient with insulinoam, the important problems are to recognize and treat hypoglycemia. This is a case report of a patient with insulinoma who underwent surgical treatment. Two years age, under the diagnosis of insulinoma, she was underwent distal pancreatectomy with splenectomy. But 3 months ago, hypoglycemic attacks recurred during fasting periods surgical intervention was performed under the diagnosis of recurrent insulinoma. She was anesthetized with thalamonal-nitrous oxide-oxygen, pancuronium, and 10 percent dextrose solution was administered throughout the operation. We monitered the level of blood sugar intermittently by using a rapie sugar analyser(Glucometer, Ames, Japan). No hypoglycemic episode during anesthesia was observed. We report the case of our anesthetic experience withan insulinoma and review the anesthetic choice and the management of the patient.
Anesthesia* ; Blood Glucose ; Coma ; Diagnosis ; Fasting ; Glucose ; Humans ; Hypoglycemia ; Incidence ; Insulinoma* ; Obesity ; Pancreas ; Pancreatectomy ; Pancuronium ; Splenectomy

Insulinomas are very rare tumors. Diagnosis of insulinoma is often delayed or misdiagnosed because of its various symptoms. We report a patient with hypoglycemic encephalopathy who had repetitive behavior changes, diagnosed as a pancreatic insulinoma. A 52 years old man was referred to a memory and dementia clinic for evaluation of his repetitive abnormal behavior changes. He has threatened his wife with violent acts and showed aggressive behaviors, but he couldn't remember when he was recovered to normal state. During the hospitalization, we noticed that his repetitive abnormal behaviors were correlated to severe hypoglycemia. After we corrected glucose level, his symptoms were disappeared. We performed an abdomen-pelvis CT scan, revealing pancreatic head tumor. After he took surgical treatment, His symptoms were fully recovered. Our case demonstrates that various neurological symptoms, such as abnormal behavior, rarely occur due to recurrent hypoglycemia in patient with insulinoma.
Amnesia* ; Dementia ; Diagnosis ; Glucose ; Head ; Hospitalization ; Humans ; Hypoglycemia ; Insulinoma* ; Memory ; Spouses ; Tomography, X-Ray Computed

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