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a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/08/06 00:51:47」(JST)

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Hyperosmolar hyperglycemic state (HHS) is a complication of diabetes mellitus in which high blood sugar results in high osmolarity without significant ketoacidosis.^[4] Symptoms include signs of dehydration, weakness, legs cramps, trouble seeing, and an altered level of consciousness.^[2] Onset is typically over days to weeks.^[3] Complications may include seizures, disseminated intravascular coagulopathy, mesenteric artery occlusion, or rhabdomyolysis.^[2]

The main risk factor is a history of diabetes mellitus type 2.^[4] Occasionally it may occur in those without a prior history of diabetes or those with diabetes mellitus type 1.^[3]^[4] Triggers include infections, stroke, trauma, certain medications, and heart attacks.^[4] Diagnosis is based on blood tests finding a blood sugar greater than 30 mmol/L (600 mg/dL), osmolarity greater than 320 mOsm/kg, and a pH above 7.3.^[2]^[3]

Initial treatment generally consists of intravenous fluids to manage dehydration, intravenous insulin in those with significant ketones, low molecular weight heparin to decrease the risk of blood clotting, and antibiotics among those in whom there is concerns of infection.^[3] The goal is a slow decline in blood sugar levels.^[3] Potassium replacement is often required as the metabolic problems are corrected.^[3] Efforts to prevent diabetic foot ulcers are also important.^[3] It typically takes a few days for the person to return to baseline.^[3]

While the exact frequently of the condition is unknown, it is relatively common.^[4]^[2] Older people are most commonly affected.^[4] The risk of death among those affected is about 15%.^[4] It was first described in the 1880s.^[4]

Signs and symptoms

Symptoms include an altered level of consciousness. Additionally it may also lead to:

Neurologic signs including focal signs such as sensory or motor impairments or focal seizures or motor abnormalities, including flaccidity, depressed reflexes, tremors or fasciculations.
Hyperviscosity and increased risk of blood clot formation

Cause

The main risk factor is a history of diabetes mellitus type 2.^[4] Occasionally it may occur in those without a prior history of diabetes or those with diabetes mellitus type 1.^[3]^[4] Triggers include infections, stroke, trauma, certain medications, and heart attacks.^[4]

Pathophysiology

Nonketotic coma is usually precipitated by an infection,^[5] myocardial infarction, stroke or another acute illness. A relative insulin deficiency leads to a serum glucose that is usually higher than 33 mmol/L (600 mg/dL), and a resulting serum osmolarity that is greater than 320 mOsm. This leads to excessive urination (more specifically an osmotic diuresis), which, in turn, leads to volume depletion and hemoconcentration that causes a further increase in blood glucose level. Ketosis is absent because the presence of some insulin inhibits hormone-sensitive lipase mediated fat tissue breakdown.

Diagnosis

Criteria

According to the consensus statement published by the American Diabetes Association, diagnostic features of HHS may include the following:^[6]^[7]

Plasma glucose level >30 mmol/L (>600 mg/dL)
Serum osmolality >320 mOsm/kg
Profound dehydration, up to an average of 9L (and therefore substantial thirst (polydipsia))
Serum pH >7.30
Bicarbonate >15 mEq/L
Small ketonuria (~+ on dipstick) and absent-to-low ketonemia (<3 mmol/L)
Some alteration in consciousness

Imaging

Cranial imaging is not used for diagnosis of this condition. However, if MRI is performed, it may show cortical restricted diffusion with unusual characteristics of reversible T2 hypointensity in the subcortical white matter.^[8]

Differential diagnosis

The major differential diagnosis is diabetic ketoacidosis (DKA). In contrast to DKA, serum glucose levels in HHS are extremely high, usually greater than 40-50 mmol/L, but an anion-gap metabolic acidosis is absent or mild. Delirium is also more common in HHS than DKA. Although traditionally DKA has been associated with Type I Diabetes, whereas HHS has been associated with Type II, HHS can be seen in people of both types. HHS also tends to have an elderly preponderance.

Management

Intravenous fluids

Treatment of HHS begins with reestablishing tissue perfusion using intravenous fluids. People with HHS can be dehydrated by 8 to 12 liters. Attempts to correct this usually take place over 24 hours with initial rates of normal saline often in the range of 1 L/h for the first few hours or until the condition stabilizes.^[9]

Electrolyte replacement

Severe potassium deficits often occur in HHS. They usually range around 350 mEq in a 70 kg person. This is generally replaced at a rate 10 mEq per hour as long as there is urinary output.^[10]

Insulin

Insulin is given to reduce blood glucose concentration; however, as it also causes the movement of potassium into cells, serum potassium levels must be sufficiently high or dangerously low blood potassium levels may result. Once potassium levels have been verified to be greater than 3.3 mEq/l, then an insulin infusion of 0.1 units/kg/hr is started.^[11]

References

^ "Hyperosmolar Hyperglycemic Nonketotic Syndrome (HHNS)". American Diabetes Association. Retrieved 6 July 2012.
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ Stoner, GD (1 May 2005). "Hyperosmolar hyperglycemic state.". American family physician. 71 (9): 1723–30. PMID 15887451.
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j ^k ^l Frank, LA; Solomon, A (2 September 2016). "Hyperglycaemic hyperosmolar state.". British journal of hospital medicine (London, England : 2005). 77 (9): C130–3. PMID 27640667. doi:10.12968/hmed.2016.77.9.C130.
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j ^k ^l ^m Pasquel, FJ; Umpierrez, GE (November 2014). "Hyperosmolar hyperglycemic state: a historic review of the clinical presentation, diagnosis, and treatment.". Diabetes care. 37 (11): 3124–31. PMID 25342831. doi:10.2337/dc14-0984.
^ Stoner, GD (May 2005). "Hyperosmolar hyperglycemic state". American Family Physician. 71 (9): 1723–30. PMID 15887451.
^ Lewis P. Rowland; Timothy A. Pedley (2010). Merritt's Neurology. Lippincott Williams & Wilkins. pp. 369–370. ISBN 978-0-7817-9186-1.
^ Magee MF, Bhatt BA (2001). "Management of decompensated diabetes. Diabetic ketoacidosis and hyperglycemic hyperosmolar syndrome.". Crit Care Clin. 17 (1): 75–106. PMID 11219236.
^ Neuroradiology 2007 Apr;49(4):299-305.
^ Tintinalli, Judith E.; Kelen, Gabor D.; Stapczynski, J. Stephan; American College of Emergency Physicians (2004). Emergency Medicine: A Comprehensive Study Guide (6th ed.). McGraw-Hill Prof Med/Tech. p. 1309. ISBN 978-0-07-138875-7.
^ Tintinalli, Kelen & Stapczynski 2004, p. 1320
^ Tintinalli, Kelen & Stapczynski 2004, p. 1310

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UpToDate Contents

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1. 成人における糖尿病性ケトアシドーシスおよび高浸透圧性高血糖状態：治療 diabetic ketoacidosis and hyperosmolar hyperglycemic state in adults treatment
2. 成人における糖尿病性ケトアシドーシスおよび高浸透圧性高血糖状態：臨床的特徴、評価、および診断 diabetic ketoacidosis and hyperosmolar hyperglycemic state in adults clinical features evaluation and diagnosis
3. 成人における糖尿病性ケトアシドーシスおよび高浸透圧性高血糖状態：疫学および病因 diabetic ketoacidosis and hyperosmolar hyperglycemic state in adults epidemiology and pathogenesis
4. 小児や青年の2型糖尿病の疫学、症状、および診断 epidemiology presentation and diagnosis of type 2 diabetes mellitus in children and adolescents
5. 小児や青年の2型糖尿病の併存疾患および合併症 comorbidities and complications of type 2 diabetes mellitus in children and adolescents

English Journal

Too much of a good thing: Part 2: Management of hyperosmolar hyperglycemic syndrome.

Blouin D.SourceDepartment of Emergency Medicine, Queen's University, Kingston, Ontario.
Canadian family physician Médecin de famille canadien.Can Fam Physician.2012 Oct;58(10):1099-100.
PMID 23064920

Coexistence of neuroleptic malignant syndrome and a hyperosmolar hyperglycemic state.

Yang CW, Lu C, Wu CC, Wen SC.SourceDepartment of Internal Medicine, Hsin Chu General Hospital, School of Medicine, National Yang-Ming University, Hsinchu City 300, Taiwan.
The American journal of emergency medicine.Am J Emerg Med.2012 Jun;30(5):833.e1-2. doi: 10.1016/j.ajem.2011.02.031. Epub 2011 Apr 22.
Neuroleptic malignant syndrome (NMS) is a rare idiosyncratic disorder characterized by muscle rigidity, hyperthermia, autonomic dysfunction, and altered consciousness. Although the incidence of NMS is low, it may be fatal if early recognition is delayed. There are a variety of precipitating factors
PMID 21514763

Stroke in a child with Adams-Oliver syndrome and mixed diabetic ketoacidosis and hyperglycemic hyperosmolar syndrome.

Nwosu BU, Adhami S, Rogol AD.SourceDivision of Endocrinology, Department of Pediatrics, University of Massachusetts Medical School, Worcester, MA 01655, USA. Benjamin.Nwosu@umassmemorial.org
Journal of pediatric endocrinology & metabolism : JPEM.J Pediatr Endocrinol Metab.2012;25(3-4):357-61.
Diabetes mellitus complicated by mixed diabetic ketoacidosis and hyperglycemic hyperosmolar syndrome presents a special challenge to physicians. There is no standard protocol for the management of mixed hyperglycemic hyperosmolar syndrome and diabetic ketoacidosis in children. The commonest cause of
PMID 22768669

Japanese Journal

非ケトン性高浸透圧性糖尿病性昏睡の治療方針 : Low-dose Dopamine療法の応用

下田雅美,山田晋也,篠田正樹,小田真理,日高充,山本勇夫,佐藤修,津金隆一
Neurologia medico-chirurgica 29(10), 890-894, 1989-10-15
… In the acute neurosurgical setting, nonketotic hyperosmolar hyperglycemic coma (NHC) is thought to be caused by cerebral dehydration therapy and administration of steroids, glycerol, or mannitol. … 1 to 5 μg/kg/min) administration in 10 patients with this syndrome. …
NAID 110002277963

Hyperosmolar hyperglycemic nonketotic syndrome. Report of 22 cases and brief review

KHARDORI R
Am J Med 77, 899-904, 1984
NAID 30008546518

Related Pictures

★リンクテーブル★

リンク元	「高浸透圧高血糖症候群」「HHS」「hyperglycemic hyperosmolar syndrome」
関連記事	「hyperosmolar」「syndrome」

「高浸透圧高血糖症候群」

Classification	ICD-10: E10-E14; ICD-9-CM: 250.2; MeSH: D006944; DiseasesDB: 29213;
External resources	eMedicine: emerg/264;

Hyperosmolar hyperglycemic state
Synonyms	Hyperosmolar hyperglycemic nonketotic coma (HHNC), hyperosmolar non-ketotic coma (HONK), nonketotic hyperosmolar coma, hyperosmolar hyperglycemic nonketotic syndrome (HHNS)
	Play media
	Video describing diabetes and its complications, including HHS.
Symptoms	Signs of dehydration, altered level of consciousness
Complications	Disseminated intravascular coagulopathy, mesenteric artery occlusion, rhabdomyolysis
Usual onset	Days to weeks
Duration	Few days
Risk factors	Infections, stroke, trauma, certain medications, heart attacks
Diagnostic method	Blood tests
Similar conditions	Diabetic ketoacidosis
Treatment	Intravenous fluids, insulin, low molecular weight heparin, antibiotics
Prognosis	~15% risk of death
Frequency	Relatively common
	[edit on Wikidata]

　　[★]

英: hyperosmolar hyperglycemic nonketotic syndrome
同: 高血糖高浸透圧症候群 hyperglycemic hyperosmolar syndrome HHS、非ケトン性高浸透圧性症候群　← 以前の呼称らしい
関: 高浸透圧性非ケトン性昏睡? hyperosmolar nonketotic coma?。糖尿病

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研修医当直御法度症例帳 p.18

2型糖尿病

誘発因子

糖尿病専門医研修ガイドブック改訂第4版 p.190

感染症：肺炎、尿路感染症、ウイルス感染
脱水：嘔吐、下痢
手術：胸部手術、腹部手術、脳外科手術
脳血管障害：脳梗塞、脳出血
薬剤：ステロイド、利尿薬、高カロリー輸液
内分泌疾患：Cushing syndrome、バセドウ病
心疾患：心筋梗塞、心不全

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