• 대한장애인치과학회지
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• 제3권1호
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• pp.22-25
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• 2007

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) characterized by severe hypoglycemia caused by inappropriate over secretion of insulin is the most common cause of hypoglycemia in early infancy. The symptoms of hypoglycemia in neonate and infancy are neonatal sepsis, respiratory difficulty, tachypnea, apnea, cyanosis, and seizure. Especially the recurrent and severe hypoglycemia within $1^{st}$ year of life is responsible for severe and irreversible brain damage. To prevent it aggressive treatment is required. Due to severe and irreversible brain damage these children frequently require anesthesia during imaging procedures such as MRI or during various dental surgical procedures. Because of frequent hypoglycemia and dental phobia in children with neurologic disorder, anesthesiologists should pay attention to patient. We report a successful anesthetic management in a patient with PHHI for dental procedures.

• 대한신경집중치료학회지
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• 제11권2호
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• pp.143-147
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• 2018

Background: Hypoglycemia is uncommon in people without diabetes. There have been only a few reports of cardiac arrest in conjunction with hypoglycemia in non-diabetic patients. Case Report: A 66-year-old man visited the emergency room with dizziness. He was a chronic alcoholic. Laboratory test showed no evidence of diabetes mellitus. Brain magnetic resonance imaging revealed a left cerebellar infarction. Abdomen computed tomography demonstrated liver cirrhosis with minimal ascites. During his hospital stay, he consumed only a small amount of food because of nausea and headache. On hospital day 4, he had a cardiac arrest after two seizure episodes. His blood glucose was 10 mg/dL. The combination of liver cirrhosis, renal failure and poor oral intake was presumed to be the causes of the severe hypoglycemia. Conclusion: We report a rare case of cardiac arrest occurring in conjunction with severe hypoglycemia in a non-diabetic patient with cerebral infarction.

• Annals of Pediatric Endocrinology and Metabolism
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• 제23권4호
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• pp.226-228
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• 2018

Various endocrine dysfunctions occur during chemotherapy, including hypoglycemia. However, reports of hypoglycemia associated with 6-mercaptopurine (6-MP) are rare. Herein, we report an 8-year-old boy with severe symptomatic hypoglycemia likely due to 6-MP during chemotherapy. He had been diagnosed with acute lymphoblastic leukemia 3 years previously and was in the maintenance chemotherapy period. Treatment included oral dexamethasone, methotrexate, and 6-MP, of which only 6-MP was administered daily. Hypoglycemic symptoms appeared mainly at dawn, and his serum glucose dropped to a minimum of 37 mg/dL. Laboratory findings showed nothing specific other than increased serum cortisol, free fatty acids, ketone, alanine aminotransferase, and aspartate aminotransferase. Under the hypothesis of hypoglycemia due to chemotherapy drugs, we changed the time of 6-MP from evening to morning and recommended him to ingest carbohydrate-rich foods before bedtime. Hypoglycemia improved dramatically, and there was no further episode during the remaining maintenance chemotherapy period. To the best of our knowledge, this is the first report of this type of hypoglycemia occurring in an Asian child including Korean.

• 대한유전성대사질환학회지
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• 제22권1호
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• pp.21-27
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• 2022

Very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency (VLCADD) leads to a defective 𝛽-oxidation, specifically during prolonged fasting, infection, or exercise. Patients with VLCADD usually suffer from cardiomyopathy, hypoketotic hypoglycemia, hepatic dysfunction, exercise intolerance, muscle pain, and rhabdomyolysis, and sometimes succumb to sudden death. VLCADD is generally classified into three phenotypes: severe early-onset cardiac and multiorgan failure, hypoketotic hypoglycemia, and later-onset episodic myopathy. Diagnostic evaluation comprises acylcarnitine analysis, genetic analysis, and VLCAD activity assay. In the acylcarnitine analysis, the key metabolites are C14:1, C14:2, C14, and C12:1. A C14:1 level >1 mmol/L strongly suggests VLCADD. Various treatment recommendations are available for this condition. Dietary management includes decreasing fat content, increasing medium-chain triglyceride levels, and decreasing fasting periods. Supplementation with L-carnitine is controversial. Triheptanoin (a seven-carbon fatty acid triglyceride) treatment demonstrates improvement of cardiac functions. Bezafibrate may improve the quality of life of patients with VLCAD.

• 대한유전성대사질환학회지
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• 제23권2호
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• pp.8-14
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• 2023

간 당원축적병 0형은 glycogen synthase 2 유전자에 부호화되어 있는 간 당원 합성효소의 결핍으로 비정상적으로 당원 생성이 되는 상염색체 열성 유전 질환이다. 당원축적병 0형의 임상 양상은 공복시에 고케톤혈증 저혈당증을 나타내고 식사후 고혈당과 고젖산혈증을 보인다. 당원축적병 0형은 현재까지 적은 수만 보고되었는데 증상이 경하거나 심한 저혈당이 드물고 또는 무증상이거나 나이가 듦에 따라 점차 증상이 사라지는 양상을 보이기 때문에 진단을 놓치는 경우가 있을 것으로 생각된다. 필수적 치료 전략은 포도당신생성을 자극하기 위해 고단백 식사, 낮동안 저혈당을 방지하기 위해서 잦은 식사 횟수, 밤 동안 천천히 포도당을 방출하기 위해 생옥수수전분가루 같은 복합 탄수화물을 먹는 것이다. 당원축적병 0형은 예후는 좋고 적절한 치료를 하면 정상적으로 성장하며 합병증도 발생하지 않는다. 성인이 될수록 심한 저혈당은 보이지 않게 되지만 지속적인 식사 관리는 필요하다.

• Asian-Australasian Journal of Animal Sciences
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• 제7권4호
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• pp.471-474
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• 1994

Five experimental groups with five adult male rats in each, were exposed to 20, 35, 40 and $45^{\circ}C$ air temperature for 50-70 minutes, and to $50^{\circ}C$ for 30-50 minutes, respectively. Food and drinking water were not permitted during the exposure. Blood samples were obtained by heart puncture immediately after the thermal treatment. All the rats were hyperthermic (p<0.01) as compared to the controls ($20^{\circ}C$). Hyperthermia was associated with hypoglycemia which was significant (p<0.01) at 45 and $50^{\circ}C$ exposures. Plasma levels of GOT and GPT declined at 35 and $40^{\circ}C$ reaching the lowest (p<0.05) level at $45^{\circ}C$, while at $50^{\circ}C$ GOT level was elevated by 45% but GPT was normal as compared to the controls. Differences between groups were significant (p<0.01) for GOT and insignificant for GPT. Hematocrit value increased significantly (p<0.01) at 45 and $50^{\circ}C$, indicating hemoconcentration. It could be concluded that severe heat stress (45 and $50^{\circ}C$) resulted in critical hyperthermia, hypoglycemia, disturbed liver function, body dehydration, and hemoconcentration leading to death.

• Journal of Yeungnam Medical Science
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• 제36권3호
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• pp.183-191
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• 2019

Some patients with type 1 and type 2 diabetes mellitus (DM) present with cognitive dysfunctions. The pathophysiology underlying this complication is not well understood. Type 1 DM has been associated with a decrease in the speed of information processing, psychomotor efficiency, attention, mental flexibility, and visual perception. Longitudinal epidemiological studies of type 1 DM have indicated that chronic hyperglycemia and microvascular disease, rather than repeated severe hypoglycemia, are associated with the pathogenesis of DM-related cognitive dysfunction. However, severe hypoglycemic episodes may contribute to cognitive dysfunction in high-risk patients with DM. Type 2 DM has been associated with memory deficits, decreased psychomotor speed, and reduced frontal lobe/executive function. In type 2 DM, chronic hyperglycemia, long duration of DM, presence of vascular risk factors (e.g., hypertension and obesity), and microvascular and macrovascular complications are associated with the increased risk of developing cognitive dysfunction. The pathophysiology of cognitive dysfunction in individuals with DM include the following: (1) role of hyperglycemia, (2) role of vascular disease, (3) role of hypoglycemia, and (4) role of insulin resistance and amyloid. Recently, some investigators have proposed that type 3 DM is correlated to sporadic Alzheimer's disease. The molecular and biochemical consequences of insulin and insulin-like growth factor resistance in the brain compromise neuronal survival, energy production, gene expression, plasticity, and white matter integrity. If patients claim that their performance is worsening or if they ask about the effects of DM on functioning, screening and assessment are recommended.

• 대한임상독성학회지
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• 제14권2호
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• pp.151-154
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• 2016

Increased plasma insulin levels are often observed in exogenous insulin overdose patients. However, plasma insulin level may decrease with time. We report a case of low plasma insulin level hypoglycemia after insulin lispro overdose. The patient was a 37-year-old man with no previous medical history who suspected insulin lispro overdose. Upon arrival, his Glasgow coma scale was 3 points and his blood sugar level (BSL) was 24 mg/dl. We found five humalog-quick-pen (insulin lispro) in his bag. There was no elevation of glucose level, despite an initial 50 ml bolus of 50% glucose and 150 cc/hr of 10% dextrose continuous intravenous infusion. He also suffered from generalized tonic-clonic seizure, which was treated with lorazepam and phenytoin. We conducted endotracheal intubation, after which he was admitted to the intensive care unit (ICU). There were recurrent events of hypoglycemia below BSL<50 mg/dl after admission. We repeatedly infused 50 ml 50% glucose 10 times and administered 1 mg of glucagon two times. The plasma insulin level was 0.2 uU/ml on initial blood sampling and 0.2 uU/ml after 5 hours. After 13 hours, his BSL stabilized but his mental status had not recovered. Diffuse brain injury was observed upon magnetic resonance imaging (MRI) and severe diffuse cerebral dysfunction was found on electroencephalography (EEG). Despite 35 days of ICU care, he died from ventilator associated pneumonia.

• 대한유전성대사질환학회지
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• 제13권1호
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• pp.43-47
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• 2013

Introduction: ALT/AST enzymes are present inside the cells. AST is found in cardiac and skeletal muscle and red blood cells but the ALT is checked mainly in the liver. In general, the rise of these two indicators shows liver damage. The usual measurements of these enzymes are used in liver function tests, but the levels of AST and ALT do not always reflect liver function. Method and Cases: 17 cases of liver dysfunction transiently were evaluated clinically, biochemically, and imaging study of sonogram in pediatric in-patients for 3 years. Result: Most common causes of transient liver dysfunction were infection, especially viral gastroenteritis, and bacterial infection interfering oral food intake. More often occurred in the children who have infant hyperbilirubinemia, positive history of mitochondrial dysfunction or hypoglycemia. Fasting study in one case of hypoglycemia patient showed reversible liver dysfunction during fasting over 20 hours fasting. Discussion: A significant increase in AST and ALT with normal bilirubin can be observed in clinically healthy people during blunt trauma, viral infection, severe pain, metabolic syndrome, fasting or accidental health screening.

• 대한유전성대사질환학회지
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• 제22권1호
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• pp.1-8
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• 2022

Long-chain fatty acid oxidation disorders (LC-FAOD) are an autosomal recessive inherited rare disease group that result in an acute metabolic crisis and chronic energy deficiency owing to the deficiency in an enzyme that converts long-chain fatty acids into energy. LC-FAOD includes carnitine palmitoyltransferase type 1 (CPT1), carnitine-acylcarnitine translocase (CACT), carnitine palmitoyltransferase type 2 (CPT2), very long-chain acyl-CoA dehydrogenase (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), and trifunctional protein (TFP) deficiencies. Common symptoms of LC-FAOD are hypoketotic hypoglycemia, cardiomyopathy, and myopathy. Depending on symptom onset, the disease can be divided as neonatal period, late infancy and early childhood, adolescence, or adult onset, but symptoms can appear at any time. The neonatal screening test (NBS) can be used to identify the characteristic plasma acylcarnitine profiles for each disease and confirmed by deficient enzyme analysis or molecular testing. Before introduction of NBS, the mortality rate of LC-FAOD was very high. With NBS implementation as routine neonatal care, the mortality rate was dramatically decreased, but severe symptoms such as rhabdomyolysis recur frequently and affect the quality of life. Triheptanoin (Dojolvi^®), the first drug for pediatric and adult patients with molecularly confirmed LC-FAOD, has recently been approved by the US Food and Drug Administration in 2020. In this review, the diagnosis of LC-FAOD and treatment including triheptanoin are summarized.