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Hypernatremic dehydration

MedGen UID:
340564
Concept ID:
C1850544
Finding
Synonym: Hypernatremic Dehydration
SNOMED CT: Hypernatremic dehydration (427784006)
 
HPO: HP:0004906

Definition

Dehydration resulting from abnormally high levels of sodium in the blood. Infants and individuals that are intubated are at highest risk. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypernatremic dehydration

Conditions with this feature

Isolated hyperchlorhidrosis
MedGen UID:
333560
Concept ID:
C1840437
Finding
Isolated hyperchlorhidrosis (HYCHL) is an autosomal recessive condition in which excessive salt wasting in sweat can result in severe infantile hyponatremic dehydration and hyperkalemia (summary by Muhammad et al., 2011).
Netherton syndrome
MedGen UID:
1802991
Concept ID:
C5574950
Disease or Syndrome
Netherton syndrome (NETH) is a rare and severe autosomal recessive skin disorder characterized by congenital erythroderma, a specific hair-shaft abnormality, and atopic manifestations with high IgE levels. Generalized scaly erythroderma is apparent at or soon after birth and usually persists. Scalp hair is sparse and brittle with a characteristic 'bamboo' shape under light microscopic examination due to invagination of the distal part of the hair shaft to its proximal part. Atopic manifestations include eczema-like rashes, atopic dermatitis, pruritus, hay fever, angioedema, urticaria, high levels of IgE in the serum, and hypereosinophilia. Life-threatening complications are frequent during the neonatal period, including hypernatremic dehydration, hypothermia, extreme weight loss, bronchopneumonia, and sepsis. During childhood, failure to thrive is common as a result of malnutrition, metabolic disorders, chronic erythroderma, persistent cutaneous infections, or enteropathy (summary by Bitoun et al., 2002).

Professional guidelines

PubMed

Zieg J, Narla D, Gonsorcikova L, Raina R
Pediatr Nephrol 2024 Feb;39(2):423-434. Epub 2023 Jul 14 doi: 10.1007/s00467-023-06080-z. PMID: 37452205
Santillanes G, Rose E
Emerg Med Clin North Am 2018 May;36(2):259-273. Epub 2018 Feb 10 doi: 10.1016/j.emc.2017.12.004. PMID: 29622321
Glick JB, Craiglow BG, Choate KA, Kato H, Fleming RE, Siegfried E, Glick SA
Pediatrics 2017 Jan;139(1) Epub 2016 Dec 20 doi: 10.1542/peds.2016-1003. PMID: 27999114

Recent clinical studies

Etiology

Johnson E, Hunt R
Curr Opin Pediatr 2019 Aug;31(4):476-481. doi: 10.1097/MOP.0000000000000791. PMID: 31188166
Sarin A, Thill A, Yaklin CW
Pediatr Ann 2019 May 1;48(5):e197-e200. doi: 10.3928/19382359-20190424-01. PMID: 31067335
Neifert MR
Pediatr Clin North Am 2001 Apr;48(2):273-97. doi: 10.1016/s0031-3955(08)70026-9. PMID: 11339153
Molaschi M, Ponzetto M, Massaia M, Villa L, Scarafiotti C, Ferrario E
J Nutr Health Aging 1997;1(3):156-60. PMID: 10995084
Abramovici MI, Singhal PC, Trachtman H
J Med 1992;23(1):17-28. PMID: 1573339

Diagnosis

Zieg J, Narla D, Gonsorcikova L, Raina R
Pediatr Nephrol 2024 Feb;39(2):423-434. Epub 2023 Jul 14 doi: 10.1007/s00467-023-06080-z. PMID: 37452205
Sarin A, Thill A, Yaklin CW
Pediatr Ann 2019 May 1;48(5):e197-e200. doi: 10.3928/19382359-20190424-01. PMID: 31067335
Santillanes G, Rose E
Emerg Med Clin North Am 2018 May;36(2):259-273. Epub 2018 Feb 10 doi: 10.1016/j.emc.2017.12.004. PMID: 29622321
Neifert MR
Pediatr Clin North Am 2001 Apr;48(2):273-97. doi: 10.1016/s0031-3955(08)70026-9. PMID: 11339153
Conley SB
Pediatr Clin North Am 1990 Apr;37(2):365-72. doi: 10.1016/s0031-3955(16)36874-2. PMID: 2184402

Therapy

Zieg J, Narla D, Gonsorcikova L, Raina R
Pediatr Nephrol 2024 Feb;39(2):423-434. Epub 2023 Jul 14 doi: 10.1007/s00467-023-06080-z. PMID: 37452205
Santillanes G, Rose E
Emerg Med Clin North Am 2018 May;36(2):259-273. Epub 2018 Feb 10 doi: 10.1016/j.emc.2017.12.004. PMID: 29622321
Cardiello V, Zecca E, Corsello M, Pianini T, Serrao F, Costa S, Cota F
Early Hum Dev 2018 Apr;119:45-50. Epub 2018 Mar 18 doi: 10.1016/j.earlhumdev.2018.03.002. PMID: 29562206
Ben-Shalom E, Toker O, Schwartz S
Isr Med Assoc J 2016 Feb;18(2):95-9. PMID: 26979001
Neifert MR
Pediatr Clin North Am 2001 Apr;48(2):273-97. doi: 10.1016/s0031-3955(08)70026-9. PMID: 11339153

Prognosis

Boskabadi H, Zakerihamidi M, Moradi A
J Matern Fetal Neonatal Med 2022 Jan;35(1):66-74. Epub 2020 Jan 14 doi: 10.1080/14767058.2020.1712698. PMID: 31937159
Tudor RM, Thompson CJ
Pituitary 2019 Jun;22(3):296-304. doi: 10.1007/s11102-018-0917-z. PMID: 30334138
Cardiello V, Zecca E, Corsello M, Pianini T, Serrao F, Costa S, Cota F
Early Hum Dev 2018 Apr;119:45-50. Epub 2018 Mar 18 doi: 10.1016/j.earlhumdev.2018.03.002. PMID: 29562206
Glick JB, Craiglow BG, Choate KA, Kato H, Fleming RE, Siegfried E, Glick SA
Pediatrics 2017 Jan;139(1) Epub 2016 Dec 20 doi: 10.1542/peds.2016-1003. PMID: 27999114
Finberg L
N Engl J Med 1973 Jul 26;289(4):196-8. doi: 10.1056/NEJM197307262890407. PMID: 4576533

Clinical prediction guides

Arora I, Juneja H, Bhandekar H, Chandankhede M
J Matern Fetal Neonatal Med 2024 Dec;37(1):2299568. Epub 2023 Dec 27 doi: 10.1080/14767058.2023.2299568. PMID: 38151267
Tanwar P, Kapoor K, Kumar A, Gangopadhyay S, Gera R
Pediatr Emerg Care 2024 Apr 1;40(4):e10-e15. Epub 2023 Aug 15 doi: 10.1097/PEC.0000000000003028. PMID: 37586360
Krzemień G, Pańczyk-Tomaszewska M, Antonowicz-Zawiślak A, Szmigielska A
Pol Merkur Lekarski 2020 Oct 23;48(287):307-311. PMID: 33130788
Cardiello V, Zecca E, Corsello M, Pianini T, Serrao F, Costa S, Cota F
Early Hum Dev 2018 Apr;119:45-50. Epub 2018 Mar 18 doi: 10.1016/j.earlhumdev.2018.03.002. PMID: 29562206
Molaschi M, Ponzetto M, Massaia M, Villa L, Scarafiotti C, Ferrario E
J Nutr Health Aging 1997;1(3):156-60. PMID: 10995084

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