ketotic hypoglycemia

ケトン性低血糖症

WordNet

1. abnormally low blood sugar usually resulting from excessive insulin or a poor diet (同)hypoglycaemia

PrepTutorEJDIC

1. 低血糖症,血糖減少症

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/02/02 21:46:45」(JST)

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Ketotic hypoglycemia is a medical term used in two ways: (1) broadly, to refer to any circumstance in which low blood glucose is accompanied by ketosis, and (2) in a much more restrictive way to refer to recurrent episodes of hypoglycemic symptoms with ketosis and, often, vomiting, in young children. The first usage refers to a pair of metabolic states (hypoglycemia plus ketosis) that can have many causes, while the second usage refers to a specific "disease" called ketotic hypoglycemia.

Hypoglycemia with ketosis: the broad sense

There are hundreds of causes of hypoglycemia. Normally, the defensive, physiological response to a falling blood glucose is reduction of insulin secretion to undetectable levels, and release of glucagon, adrenaline, and other counterregulatory hormones. This shift of hormones initiates glycogenolysis and gluconeogenesis in the liver, and lipolysis in adipose tissue. Lipids are metabolized to triglycerides, in turn to fatty acids, which are transformed in the mitochondria of liver and kidney cells to the ketone bodies— acetoacetate, beta-hydroxybutyrate, and acetone. Ketones can be used by the brain as an alternate fuel when glucose is scarce. A high level of ketones in the blood, ketosis, is thus a normal response to hypoglycemia in healthy people of all ages.

The presence or absence of ketosis is therefore an important clue to the cause of hypoglycemia in an individual patient. Absence of ketosis ("nonketotic hypoglycemia") most often indicates excessive insulin as the cause of the hypoglycemia. Less commonly, it may indicate a fatty acid oxidation disorder.

Ketotic hypoglycemia: the "disease"

Ketotic hypoglycemia more commonly refers to a common but mysterious "disease" of recurrent hypoglycemic symptoms with ketosis in young children. The cause and the homogeneity of the condition remain uncertain, but a characteristic presentation, precipitating factors, diagnostic test results, treatment, and natural history can be described. It remains one of the more common causes of hypoglycemia in the age range.

Clinical features

The typical patient with ketotic hypoglycemia is a young child between the ages of 10 months and 4 years. Episodes nearly always occur in the morning after an overnight fast, often one that is longer than usual. Symptoms include those of neuroglycopenia, ketosis, or both. The neuroglycopenic symptoms usually include lethargy and malaise, but may include unresponsiveness or seizures. The principal symptoms of ketosis are anorexia, abdominal discomfort, and nausea, sometimes progressing to vomiting.

If severe, parents usually take the child to a local emergency department, where blood is drawn. The glucose is usually found to be between 35 and 60 mg/dl (1.8-3.1 mMol/L). The total CO₂ is usually somewhat low as well, (14-19 mMol/L is typical), and if urine is obtained, high levels of ketones are discovered. Ketones can also be measured in the blood at the bedside (Medisense glucometer). Other routine tests are normal. If given intravenous fluids with saline and dextrose, the child improves dramatically and is usually restored to normal health within a few hours.

A first episode is usually attributed to a viral infection or acute gastroenteritis. However, in most of these children one or more additional episodes recur over next few years and become immediately recognizable to the parents. In mild cases, carbohydrates and a few hours of sleep will be enough to end the symptoms.

Precipitating factors, conditions that trigger an episode, may include extended fasting (e.g., missing supper the night before), a low carbohydrate intake the previous day (e.g., a hot dog without a bun), or stress such as a viral infection. Most children affected by ketotic hypoglycemia have a slender build, many with a weight percentile below height percentile, though without other evidence of malnutrition. Overweight children are rarely affected.

Diagnosis

The diagnosis is based on a combination of typical clinical features and exclusion by a pediatric endocrinologist of other causes of "hypoglycemia with ketosis," especially growth hormone deficiency, hypopituitarism, adrenal insufficiency, and identifiable inborn errors of metabolism such as organic acidoses.

The most useful diagnostic tests include measurement of insulin, growth hormone, cortisol, and lactic acid at the time of the hypoglycemia. Plasma acylcarnitine levels and urine organic acids exclude some of the important metabolic diseases. When the episodes are recurrent or severe, the definitive test is a hospitalization for a supervised diagnostic fast. This usually demonstrates "accelerated fasting"-- a shorter time until the glucose begins to fall, but normal metabolic and counterregulatory responses as the glucose falls. As the glucose reaches hypoglycemic levels, the insulin is undetectable, counterregulatory hormones, fatty acids, and ketones are high, and glucagon injection elicits no rise of glucose.

Treatment

Once ketotic hypoglycemia is suspected and other conditions excluded, appropriate treatment reduces the frequency and duration of episodes. Extended fasts should be avoided. The child should be given a bedtime snack of carbohydrates and should be awakened and fed after the usual duration of sleep. If the child is underweight, a daily nutritional supplement may be recommended. Raw cornstarch dissolved in a beverage helps individuals with hypoglycemia, especially that caused by Glycogen Storage Disease, sustain their blood sugars for longer periods of time and may be given at bedtime.

If a spell begins, carbohydrates and fluids should be given promptly. If vomiting prevents this, the child should be taken to the local emergency department for a few hours of intravenous saline and dextrose. This treatment is often expedited by supplying the parents with a letter describing the condition and recommended treatment.

Natural history

Children "outgrow" ketotic hypoglycemia, presumably because fasting tolerance improves as body mass increases. In most the episodes become milder and more infrequent by 4 to 5 years of age and rarely occur after age 9. Onset of hypoglycemia with ketosis after age 5 or persistence after age 7 should elicit referral and an intensive search for a more specific disease.

Is ketotic hypoglycemia a disease?

Despite much investigation for fifty years, it has not been possible to demonstrate any metabolic difference that sets these children apart from the normal population except the shortened fasting tolerance. It has been proposed that this condition simply represents the extreme edge of the normal population in terms of tolerance for fasting and ability to maintain normoglycemia. It is also possible that some children given this diagnosis have still-undiscovered defects of metabolism which will eventually be identified.

References

UpToDate Contents

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• 1. 幼児​および​小児​における​低血糖症​の​病因​ etiology of hypoglycemia in infants and children
• 2. 乳児​および​小児​における​低血糖​に​対​する​アプローチ​ approach to hypoglycemia in infants and children
• 3. 尿素​サイクル​障害​：​臨床的特徴​および​診断​ urea cycle disorders clinical features and diagnosis
• 4. 肝​グリコーゲン​合成酵素欠損症​（​糖原病​0​型​）​ liver glycogen synthase deficiency glycogen storage disease 0
• 5. 小児​における​急性中毒性代謝性脳症​ acute toxic metabolic encephalopathy in children

English Journal

• Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California.

• Gallant NM, Leydiker K, Tang H, Feuchtbaum L, Lorey F, Puckett R, Deignan JL, Neidich J, Dorrani N, Chang E, Barshop BA, Cederbaum SD, Abdenur JE, Wang RY.SourceDepartment of Pediatrics, University of California at Los Angeles, Los Angeles, CA, USA; Department of Human Genetics, University of California at Los Angeles, Los Angeles, CA, USA.
• Molecular genetics and metabolism.Mol Genet Metab.2012 May;106(1):55-61. Epub 2012 Feb 9.
• BACKGROUND: Short-chain acyl-CoA dehydrogenase deficiency (SCADD) is an autosomal recessive inborn error of mitochondrial fatty acid oxidation with highly variable biochemical, genetic, and clinical characteristics. SCADD has been associated with accumulation of butyryl-CoA byproducts, including but
• PMID 22424739

• Hypoglycemia-induced hemichorea in a patient with Fahr's syndrome.

• Paraskevas GP, Vlachos GS, Vassilopoulou S, Anagnostou E, Spengos K, Zis V.Source1st Department of Neurology, Eginition Hospital, National University of Athens, 72-74 Vassilissis Sophias Ave., 11528, Athens, Greece, geoprskvs44@gmail.com.
• Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology.Neurol Sci.2012 Apr 24. [Epub ahead of print]
• Non-ketotic hyperglycemia may be a cause of hemiballism-hemichorea. We present an elderly female type II diabetic patient with right-sided hemiballism-hemichorea of acute onset during hypoglycemia following insulin overtreatment of non-ketotic hyperglycemia. Brain computerized tomography and magneti
• PMID 22526769

Japanese Journal

• 福山型先天性筋ジストロフィーにおける糖代謝

• 小峯 聡,石井 かやの,池谷 紀代子 [他],齋藤 加代子,大澤 眞木子
• 東京女子医科大学雑誌 83(E1), E25-E35, 2013-01-31
• 福山型先天性筋ジストロフィー (FCMD)は、日本では、小児期の筋ジストロフィーの中で2番目に最も多く見られる。FCMDには、長年の診療の中で本症患児の病状を脅かす問題点が多々認められている。我々は、FCMDの患者にケトン性低血糖にしばしば遭遇する。ケトン性低血糖は一般的に幼児期の健常児に認められるが、その機序は、FCMDでは明確にされていない。この研究は、FCMDにおける低血糖の機序を明らかにす …
• NAID 110009559429

• Children's toxicology from bench to bed - Liver Injury (1) : Drug-induced metabolic disturbance : Toxicity of 5-FU for pyrimidine metabolic disorders and pivalic acid for carnitine metabolism

• Ito Tetsuya
• Journal of toxicological sciences 34(Special Issue II), SP217-SP222, 2009-07
• … Because of this, long term administration of such antibiotics could induce depletion of carnitine from the body and lead to low ketotic hypoglycemia, convulsion and consciousness disturbance. …
• NAID 110007337718

• Ketotic hypoglycemia in patients with allergic diseases

• MATSUBARA TOMOYO,YOSHITOMI TOMOMI,KANEKO MIHO,ICHIYAMA TAKASHI,FURUKAWA SUSUMU
• Pediatrics international : official journal of the Japan Pediatric Society 45(6), 653-655, 2003-12-01
• NAID 10016211372

Related Links

• Ketotic Hypoglycemia Symptoms | Hypoglycemia

Ketotic Hypoglycemia and recognizing the signs to treat early ... Ketotic Hypoglycemia is a condition that in broader terms refers to the low blood sugar accompanied with ketotis, however, it is more specifically used to describe a ...

• Ketotic Hypoglycemia, Low Blood Sugar with toddler’s ...

Ketotic hypoglycemia, also known as toddler’s hypoglycaemia, describes periods of low blood glucose levels (hypoglycemia) that is accompanied by ketosis.

Related Pictures

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★リンクテーブル★

リンク元	「ケトン性低血糖症」「ケトン血性低血糖症」
関連記事	「ketotic」

「ケトン性低血糖症」

　　[★]

英

ketotic hypoglycemia

関

アセトン血性嘔吐症、低血糖症

概念

• 比較的やせ型の児が、感冒や精神的ストレスにより食事が取れないために糖の補給ができなくなり、ケトーシスを来して元気がなくなり嘔吐を発症する疾患。幼児期～児童初期にみられる。

疫学

• 小児の低血糖症の中で最も多く認められる(全体の50%)。
• 身体的には未熟児、SFD児、身体発育不良児に多い。
• 2-5歳に最も多い
• 男女比は2：1で男児に多い。
• 加齢とともに発作頻度は減少。10歳くらいまでに消失

病因

• 糖新生の基質不足や糖新生系代謝の未熟性

• 糖新生能力が低い：小児では肝のグリコーゲンは数時間で枯渇。筋から動員される糖原性アミノ酸からアラニンとなり糖新生されるが、小児では糖の補充が間に合わないことがある。

誘因

• 摂食不良・高脂肪食

• 摂食が制限されているときに起こるので、朝に起きやすいし、前日の夕食を抜いていればなおさら

病態生理

• 脂肪分解亢進　→　ケトン体増加　＋　低血糖

症状

• 悪心・嘔吐、脱力感、全身倦怠感、無表情、会話の停滞、集中力減退、速脈、顔面蒼白、歩行障害、嗜眠、意識障害、けいれん　(SPE.210)

検査

• ケトン負荷：ケトン食で低血糖発作
• グルカゴン負荷：低血糖発作時にグルカゴン負荷に反応しない

血液検査

• 血糖：低値
• グルカゴン：高値

尿検査

• ケトン尿

治療

• ブドウ糖静注

予防

• 食事回数の増加、誘発因子(感染、ストレス)を避ける

鑑別

YN.D-107

		ケトン性低血糖症	アセトン血性嘔吐症
発作時	ケトーシス	＋＋＋	＋＋＋
	低血糖症	＋＋	±～－
	痙攣	＋	－
非発作時血糖値		正常	正常
発作の誘因		摂食不良・高脂肪食	ストレス・感染
ケトン食負荷		発症	非発症
好発年齢		約1-5歳	約2-10歳
出産時状況		未熟児、SFD児に多い
予防		高炭水化物の摂取	ストレス回避

参考

uptodate

• 1. [charged] 幼児および小児における低血糖症の病因 - uptodate [1]
• 2. [charged] 乳児および小児における低血糖に対するアプローチ - uptodate [2]

国試

• 104I029

「ケトン血性低血糖症」

　　[★]

英

ketotic hypoglycemia

関

アセトン血性嘔吐症

「ketotic」

　　[★]

• adj.

• ケトーシスの

関

ketosis