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Hypoglycemic coma

MedGen UID:
5710
Concept ID:
C0020617
Disease or Syndrome
Synonyms: Coma associated with hypoglycemia; Coma, hypoglycemic; Loss of consciousness due to hypoglycemia
SNOMED CT: Coma due to hypoglycemia (267384006); Hypoglycemic coma (267384006)
 
HPO: HP:0001325

Definition

Coma induced by low blood sugar. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypoglycemic coma

Conditions with this feature

Multiple acyl-CoA dehydrogenase deficiency
MedGen UID:
75696
Concept ID:
C0268596
Disease or Syndrome
Multiple acyl-CoA dehydrogenase deficiency (MADD) represents a clinical spectrum in which presentations can be divided into type I (neonatal onset with congenital anomalies), type II (neonatal onset without congenital anomalies), and type III (late onset). Individuals with type I or II MADD typically become symptomatic in the neonatal period with severe metabolic acidosis, which may be accompanied by profound hypoglycemia and hyperammonemia. Many affected individuals die in the newborn period despite metabolic treatment. In those who survive the neonatal period, recurrent metabolic decompensation resembling Reye syndrome and the development of hypertrophic cardiomyopathy can occur. Congenital anomalies may include dysmorphic facial features, large cystic kidneys, hypospadias and chordee in males, and neuronal migration defects (heterotopias) on brain MRI. Individuals with type III MADD, the most common presentation, can present from infancy to adulthood. The most common symptoms are muscle weakness, exercise intolerance, and/or muscle pain, although metabolic decompensation with episodes of rhabdomyolysis can also be seen. Rarely, individuals with late-onset MADD (type III) may develop severe sensory neuropathy in addition to proximal myopathy.
Hyperinsulinism-hyperammonemia syndrome
MedGen UID:
376153
Concept ID:
C1847555
Disease or Syndrome
Congenital hyperinsulinism is a condition that causes individuals to have abnormally high levels of insulin. Insulin is a hormone that helps control levels of blood glucose, also called blood sugar. People with this condition have frequent episodes of low blood glucose (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, or difficulty feeding. Repeated episodes of low blood glucose increase the risk for serious complications such as breathing difficulties, seizures, intellectual disability, vision loss, brain damage, and coma.\n\nThe severity of congenital hyperinsulinism varies widely among affected individuals, even among members of the same family. About 60 percent of infants with this condition experience a hypoglycemic episode within the first month of life. Other affected children develop hypoglycemia by early childhood. Unlike typical episodes of hypoglycemia, which occur most often after periods without food (fasting) or after exercising, episodes of hypoglycemia in people with congenital hyperinsulinism can also occur after eating.
Hyperinsulinemic hypoglycemia, familial, 4
MedGen UID:
400646
Concept ID:
C1864948
Disease or Syndrome
Congenital hyperinsulinism is a condition that causes individuals to have abnormally high levels of insulin. Insulin is a hormone that helps control levels of blood glucose, also called blood sugar. People with this condition have frequent episodes of low blood glucose (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, or difficulty feeding. Repeated episodes of low blood glucose increase the risk for serious complications such as breathing difficulties, seizures, intellectual disability, vision loss, brain damage, and coma.\n\nThe severity of congenital hyperinsulinism varies widely among affected individuals, even among members of the same family. About 60 percent of infants with this condition experience a hypoglycemic episode within the first month of life. Other affected children develop hypoglycemia by early childhood. Unlike typical episodes of hypoglycemia, which occur most often after periods without food (fasting) or after exercising, episodes of hypoglycemia in people with congenital hyperinsulinism can also occur after eating.
Hyperinsulinism due to INSR deficiency
MedGen UID:
355335
Concept ID:
C1864952
Disease or Syndrome
The severity of congenital hyperinsulinism varies widely among affected individuals, even among members of the same family. About 60 percent of infants with this condition experience a hypoglycemic episode within the first month of life. Other affected children develop hypoglycemia by early childhood. Unlike typical episodes of hypoglycemia, which occur most often after periods without food (fasting) or after exercising, episodes of hypoglycemia in people with congenital hyperinsulinism can also occur after eating.\n\nCongenital hyperinsulinism is a condition that causes individuals to have abnormally high levels of insulin. Insulin is a hormone that helps control levels of blood glucose, also called blood sugar. People with this condition have frequent episodes of low blood glucose (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, or difficulty feeding. Repeated episodes of low blood glucose increase the risk for serious complications such as breathing difficulties, seizures, intellectual disability, vision loss, brain damage, and coma.
Hyperinsulinism due to glucokinase deficiency
MedGen UID:
355435
Concept ID:
C1865290
Disease or Syndrome
Congenital hyperinsulinism is a condition that causes individuals to have abnormally high levels of insulin. Insulin is a hormone that helps control levels of blood glucose, also called blood sugar. People with this condition have frequent episodes of low blood glucose (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, or difficulty feeding. Repeated episodes of low blood glucose increase the risk for serious complications such as breathing difficulties, seizures, intellectual disability, vision loss, brain damage, and coma.\n\nThe severity of congenital hyperinsulinism varies widely among affected individuals, even among members of the same family. About 60 percent of infants with this condition experience a hypoglycemic episode within the first month of life. Other affected children develop hypoglycemia by early childhood. Unlike typical episodes of hypoglycemia, which occur most often after periods without food (fasting) or after exercising, episodes of hypoglycemia in people with congenital hyperinsulinism can also occur after eating.
3-hydroxy-3-methylglutaryl-CoA synthase deficiency
MedGen UID:
414399
Concept ID:
C2751532
Disease or Syndrome
Mitochondrial HMG-CoA synthase deficiency (HMGCS2D) is an inherited metabolic disorder caused by a defect in the enzyme that regulates the formation of ketone bodies. Patients present with hypoketotic hypoglycemia, encephalopathy, and hepatomegaly, usually precipitated by an intercurrent infection or prolonged fasting (summary by Aledo et al., 2006).
Hyperinsulinemic hypoglycemia, familial, 1
MedGen UID:
419505
Concept ID:
C2931832
Disease or Syndrome
Familial hyperinsulinism, also referred to as congenital hyperinsulinism, nesidioblastosis, or persistent hyperinsulinemic hypoglycemia of infancy (PPHI), is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur (Thornton et al., 1998). Genetic Heterogeneity of Hyperinsulinemic Hypoglycemia HHF2 (601820) is caused by mutation in the KCNJ11 gene (600937) on chromosome 11p15. HHF3 (602485) is caused by mutation in the glucokinase gene (GCK; 138079) on chromosome 7p13. HHF4 (609975) is caused by mutation in the HADH gene (601609) on chromosome 4q25. HHF5 (609968) is caused by mutation in the insulin receptor gene (INSR; 147670) on chromosome 19p13. HHF6 (606762) is caused by mutation in the GLUD1 gene (138130) on chromosome 10q23. HHF7 (610021) is caused by mutation in the SLC16A1 (600682) on chromosome 1p13. There is evidence of further genetic heterogeneity of HHF.
Glucocorticoid deficiency 4
MedGen UID:
766501
Concept ID:
C3553587
Disease or Syndrome
Familial glucocorticoid deficiency (GCCD) is a rare autosomal recessive disorder characterized by an inability of the adrenal cortex to produce cortisol in response to stimulation by adrenocorticotropic hormone (ACTH). Affected individuals typically present within the first few months of life with symptoms related to cortisol deficiency, including failure to thrive, recurrent illnesses or infections, hypoglycemia, convulsions, and shock. The disease is life-threatening if untreated (summary by Meimaridou et al., 2012). For a discussion of genetic heterogeneity of familial glucocorticoid deficiency, see GCCD1 (202200).

Professional guidelines

PubMed

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Eur J Paediatr Neurol 2024 Mar;49:60-65. Epub 2024 Feb 7 doi: 10.1016/j.ejpn.2024.02.003. PMID: 38377647
Bramlage P, Tittel SR, Müther S, Reinhart-Steininger B, Haberland H, Khodaverdi S, Zimny S, Ohlenschläger U, Lanzinger S, Haak T
Acta Diabetol 2022 Nov;59(11):1453-1460. Epub 2022 Aug 7 doi: 10.1007/s00592-022-01939-3. PMID: 35933650
Zhao HL, Tong PCY, Chan JCN
Nestle Nutr Workshop Ser Clin Perform Programme 2006;11:15-29. doi: 10.1159/000094399. PMID: 16820728

Recent clinical studies

Etiology

Li Y, Xu C, Li F, Yan Z, Ye S, Ma J, Wen J
J Cardiothorac Surg 2022 Dec 18;17(1):321. doi: 10.1186/s13019-022-02093-1. PMID: 36528774Free PMC Article
Bouyaknouden D, Peddada TN, Ravishankar N, Fatima S, Fong-Isariyawongse J, Gilmore EJ, Lee JW, Struck AF, Gaspard N; CCEMRC
Neurocrit Care 2022 Aug;37(1):273-280. Epub 2022 Apr 18 doi: 10.1007/s12028-022-01495-2. PMID: 35437670
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Zhao HL, Tong PCY, Chan JCN
Nestle Nutr Workshop Ser Clin Perform Programme 2006;11:15-29. doi: 10.1159/000094399. PMID: 16820728
Tyni T, Pihko H
Acta Paediatr 1999 Mar;88(3):237-45. doi: 10.1080/08035259950169954. PMID: 10229030

Diagnosis

Li Y, Xu C, Li F, Yan Z, Ye S, Ma J, Wen J
J Cardiothorac Surg 2022 Dec 18;17(1):321. doi: 10.1186/s13019-022-02093-1. PMID: 36528774Free PMC Article
Bouyaknouden D, Peddada TN, Ravishankar N, Fatima S, Fong-Isariyawongse J, Gilmore EJ, Lee JW, Struck AF, Gaspard N; CCEMRC
Neurocrit Care 2022 Aug;37(1):273-280. Epub 2022 Apr 18 doi: 10.1007/s12028-022-01495-2. PMID: 35437670
Santra G, De D, Phaujdar S, Rudra A, Dutta PS
J Assoc Physicians India 2012 May;60:48-51. PMID: 23029725
Tyni T, Pihko H
Acta Paediatr 1999 Mar;88(3):237-45. doi: 10.1080/08035259950169954. PMID: 10229030
Jordan RM
Med Clin North Am 1983 Nov;67(6):1193-213. doi: 10.1016/s0025-7125(16)31149-x. PMID: 6415353

Therapy

Friebe L, Freitag MT, Braun M, Nicolas G, Bauman A, Bushnell D, Christ E, Wild D
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Otsuka T, Okada Y, Torimoto K, Tanaka Y
Intern Med 2018 Oct 15;57(20):2923-2927. Epub 2018 May 18 doi: 10.2169/internalmedicine.0535-17. PMID: 29780121Free PMC Article
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Basch E, Gabardi S, Ulbricht C
Am J Health Syst Pharm 2003 Feb 15;60(4):356-9. doi: 10.1093/ajhp/60.4.356. PMID: 12625217
Shapiro AM, Lakey JR, Ryan EA, Korbutt GS, Toth E, Warnock GL, Kneteman NM, Rajotte RV
N Engl J Med 2000 Jul 27;343(4):230-8. doi: 10.1056/NEJM200007273430401. PMID: 10911004

Prognosis

Bouyaknouden D, Peddada TN, Ravishankar N, Fatima S, Fong-Isariyawongse J, Gilmore EJ, Lee JW, Struck AF, Gaspard N; CCEMRC
Neurocrit Care 2022 Aug;37(1):273-280. Epub 2022 Apr 18 doi: 10.1007/s12028-022-01495-2. PMID: 35437670
Lu Z, Liu J, He Q, Chakraborty A, Zhu T
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Pharmacoepidemiol Drug Saf 2013 Dec;22(12):1326-35. Epub 2013 Oct 21 doi: 10.1002/pds.3534. PMID: 24150837Free PMC Article
Tyni T, Pihko H
Acta Paediatr 1999 Mar;88(3):237-45. doi: 10.1080/08035259950169954. PMID: 10229030
Klatt EC, Beatie C, Noguchi TT
Am J Forensic Med Pathol 1988 Jun;9(2):122-5. doi: 10.1097/00000433-198806000-00007. PMID: 3381790

Clinical prediction guides

Friebe L, Freitag MT, Braun M, Nicolas G, Bauman A, Bushnell D, Christ E, Wild D
J Nucl Med 2024 Feb 1;65(2):228-235. doi: 10.2967/jnumed.123.265894. PMID: 38164592
Bouyaknouden D, Peddada TN, Ravishankar N, Fatima S, Fong-Isariyawongse J, Gilmore EJ, Lee JW, Struck AF, Gaspard N; CCEMRC
Neurocrit Care 2022 Aug;37(1):273-280. Epub 2022 Apr 18 doi: 10.1007/s12028-022-01495-2. PMID: 35437670
Languren G, Montiel T, Julio-Amilpas A, Massieu L
Neurochem Int 2013 Oct;63(4):331-43. Epub 2013 Jul 20 doi: 10.1016/j.neuint.2013.06.018. PMID: 23876631
Shapiro AM, Lakey JR, Ryan EA, Korbutt GS, Toth E, Warnock GL, Kneteman NM, Rajotte RV
N Engl J Med 2000 Jul 27;343(4):230-8. doi: 10.1056/NEJM200007273430401. PMID: 10911004
Siesjö BK
Crit Care Med 1988 Oct;16(10):954-63. doi: 10.1097/00003246-198810000-00006. PMID: 3048896

Recent systematic reviews

Liu Y, Zhang M, Yang X, Zhang M, Fan Z, Li Y, Wang T, Chen P
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