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Postural hypotension with compensatory tachycardia

MedGen UID:
376790
Concept ID:
C1850438
Finding
HPO: HP:0005307

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPostural hypotension with compensatory tachycardia

Conditions with this feature

Hereditary insensitivity to pain with anhidrosis
MedGen UID:
6915
Concept ID:
C0020074
Disease or Syndrome
NTRK1 congenital insensitivity to pain with anhidrosis (NTRK1-CIPA) is characterized by insensitivity to pain, anhidrosis (the inability to sweat), and intellectual disability. The ability to sense all pain (including visceral pain) is absent, resulting in repeated injuries including: oral self-mutilation (biting of tongue, lips, and buccal mucosa); biting of fingertips; bruising, scarring, and infection of the skin; multiple bone fractures (many of which fail to heal properly); and recurrent joint dislocations resulting in joint deformity. Sense of touch, vibration, and position are normal. Anhidrosis predisposes to recurrent febrile episodes that are often the initial manifestation of NTRK1-CIPA. Hypothermia in cold environments also occurs. Intellectual disability of varying degree is observed in most affected individuals; hyperactivity and emotional lability are common.

Professional guidelines

PubMed

Arnold AC, Ng J, Lei L, Raj SR
Can J Cardiol 2017 Dec;33(12):1524-1534. Epub 2017 Sep 14 doi: 10.1016/j.cjca.2017.09.008. PMID: 29102451Free PMC Article

Recent clinical studies

Etiology

Wagoner AL, Shaltout HA, Fortunato JE, Diz DI
Am J Physiol Heart Circ Physiol 2016 Feb 1;310(3):H416-25. Epub 2015 Nov 25 doi: 10.1152/ajpheart.00583.2015. PMID: 26608337Free PMC Article
Thieben MJ, Sandroni P, Sletten DM, Benrud-Larson LM, Fealey RD, Vernino S, Lennon VA, Shen WK, Low PA
Mayo Clin Proc 2007 Mar;82(3):308-13. doi: 10.4065/82.3.308. PMID: 17352367
Potocka-Plazak K, Plazak W
Aging (Milano) 2001 Oct;13(5):378-84. doi: 10.1007/BF03351506. PMID: 11820711
Diehl RR, Linden D, Chalkiadaki A, Diehl A
J Auton Nerv Syst 1999 May 28;76(2-3):159-66. doi: 10.1016/s0165-1838(99)00013-2. PMID: 10412840
Louis WJ, McNeil JJ, Drummer OH
Drugs 1984;28 Suppl 2:16-34. doi: 10.2165/00003495-198400282-00003. PMID: 6151889

Diagnosis

Arnold AC, Ng J, Lei L, Raj SR
Can J Cardiol 2017 Dec;33(12):1524-1534. Epub 2017 Sep 14 doi: 10.1016/j.cjca.2017.09.008. PMID: 29102451Free PMC Article
Thieben MJ, Sandroni P, Sletten DM, Benrud-Larson LM, Fealey RD, Vernino S, Lennon VA, Shen WK, Low PA
Mayo Clin Proc 2007 Mar;82(3):308-13. doi: 10.4065/82.3.308. PMID: 17352367
Convertino VA
J Gravit Physiol 2002 Dec;9(2):1-13. PMID: 14638455
Potocka-Plazak K, Plazak W
Aging (Milano) 2001 Oct;13(5):378-84. doi: 10.1007/BF03351506. PMID: 11820711
Diehl RR, Linden D, Chalkiadaki A, Diehl A
J Auton Nerv Syst 1999 May 28;76(2-3):159-66. doi: 10.1016/s0165-1838(99)00013-2. PMID: 10412840

Therapy

Convertino VA
J Gravit Physiol 2002 Dec;9(2):1-13. PMID: 14638455
Diehl RR, Linden D, Chalkiadaki A, Diehl A
J Auton Nerv Syst 1999 May 28;76(2-3):159-66. doi: 10.1016/s0165-1838(99)00013-2. PMID: 10412840
Fulco CS, Cymerman A, Reeves JT, Rock PB, Trad LA, Young PM
Aviat Space Environ Med 1989 Nov;60(11):1049-55. PMID: 2818394
Loon NR, Wilcox CS, Folger W
Am J Med 1986 Dec;81(6):1101-4. doi: 10.1016/0002-9343(86)90419-5. PMID: 3799644
Rosen SG, Cryer PE
Am J Med 1982 May;72(5):847-50. doi: 10.1016/0002-9343(82)90559-9. PMID: 7081280

Prognosis

Wagoner AL, Shaltout HA, Fortunato JE, Diz DI
Am J Physiol Heart Circ Physiol 2016 Feb 1;310(3):H416-25. Epub 2015 Nov 25 doi: 10.1152/ajpheart.00583.2015. PMID: 26608337Free PMC Article
Louis WJ, McNeil JJ, Drummer OH
Drugs 1984;28 Suppl 2:16-34. doi: 10.2165/00003495-198400282-00003. PMID: 6151889

Clinical prediction guides

Wagoner AL, Shaltout HA, Fortunato JE, Diz DI
Am J Physiol Heart Circ Physiol 2016 Feb 1;310(3):H416-25. Epub 2015 Nov 25 doi: 10.1152/ajpheart.00583.2015. PMID: 26608337Free PMC Article
Lehmann M, Petersen KG, Khalaf AN
Klin Wochenschr 1991 Nov 26;69(19):872-9. doi: 10.1007/BF01649561. PMID: 1812316
Louis WJ, McNeil JJ, Drummer OH
Drugs 1984;28 Suppl 2:16-34. doi: 10.2165/00003495-198400282-00003. PMID: 6151889

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