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Increased circulating renin level

MedGen UID:
66818
Concept ID:
C0240783
Finding
Synonyms: Elevated plasma renin; Hyperreninemia; Increased plasma renin; Increased serum renin
 
HPO: HP:0000848

Definition

An increased level of renin in the blood. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIncreased circulating renin level

Conditions with this feature

Congenital secretory diarrhea, chloride type
MedGen UID:
78631
Concept ID:
C0267662
Disease or Syndrome
Congenital secretory chloride diarrhea is an autosomal recessive form of severe chronic diarrhea characterized by excretion of large amounts of watery stool containing high levels of chloride, resulting in dehydration, hypokalemia, and metabolic alkalosis. The electrolyte disorder resembles the renal disorder Bartter syndrome (see 607364), except that chloride diarrhea is not associated with calcium level abnormalities (summary by Choi et al., 2009). Genetic Heterogeneity of Diarrhea Other forms of diarrhea include DIAR2 (251850), caused by mutation in the MYO5B gene (606540) on 18q21; DIAR3 (270420), caused by mutation in the SPINT2 gene (605124) on 19q13; DIAR4 (610370), caused by mutation in the NEUROG3 gene (604882) on 10q21; DIAR5 (613217), caused by mutation in the EPCAM gene (185535) on 2p21; DIAR6 (614616), caused by mutation in the GUCY2C gene (601330) on 12p12; DIAR7 (615863) caused by mutation in the DGAT1 gene (604900) on 8q24; DIAR8 (616868), caused by mutation in the SLC9A3 gene (182307) on 5p15; DIAR9 (618168), caused by mutation in the WNT2B gene (601968) on 1p13; DIAR10 (618183), caused by mutation in the PLVAP gene (607647) on 19p13; DIAR11 (618662), caused by deletion of the intestine critical region (ICR) on chromosome 16p13, resulting in loss of expression of the flanking gene PERCC1 (618656); DIAR12 (619445), caused by mutation in the STX3 gene (600876) on 11q12; and DIAR13 (620357), caused by mutation in the ACSL5 gene (605677) on chromosome 10q25.
Corticosterone 18-monooxygenase deficiency
MedGen UID:
82784
Concept ID:
C0268293
Disease or Syndrome
CMO type I deficiency is an autosomal recessive disorder caused by a defect in the penultimate biochemical step of aldosterone biosynthesis, the 18-hydroxylation of corticosterone (B) to 18-hydroxycorticosterone (18-OHB). This enzymatic defect results in decreased aldosterone and salt-wasting. In CMO I deficiency, aldosterone is undetectable, whereas its immediate precursor, 18-OHB, is low or normal. These patients have an increased ratio of corticosterone to 18-OHB (Portrat-Doyen et al., 1998). The CYP11B2 gene product also catalyzes the final step in aldosterone biosynthesis: the 18-oxidation of 18-OHB to aldosterone. A defect in that enzymatic step results in CMO type II deficiency (610600), an allelic disorder with an overlapping phenotype but distinct biochemical features. In CMO II deficiency, aldosterone can be low or normal, but at the expense of increased secretion of 18-OHB. These patients have a low ratio of corticosterone to 18-OHB (Portrat-Doyen et al., 1998).
Familial hypokalemic alkalosis, Gullner type
MedGen UID:
78677
Concept ID:
C0268444
Disease or Syndrome
Familial hypokalemia-hypomagnesemia
MedGen UID:
75681
Concept ID:
C0268450
Disease or Syndrome
Gitelman syndrome (GTLMNS) is an autosomal recessive renal tubular salt-wasting disorder characterized by hypokalemic metabolic alkalosis with hypomagnesemia and hypocalciuria. It is the most common renal tubular disorder among Caucasians (prevalence of 1 in 40,000). Most patients have onset of symptoms as adults, but some present in childhood. Clinical features include transient periods of muscle weakness and tetany, abdominal pains, and chondrocalcinosis (summary by Glaudemans et al., 2012). Gitelman syndrome is sometimes referred to as a mild variant of classic Bartter syndrome (607364). For a discussion of genetic heterogeneity of Bartter syndrome, see 607364.
Autosomal dominant hypocalcemia 1
MedGen UID:
87438
Concept ID:
C0342345
Disease or Syndrome
Autosomal dominant hypocalcemia-1 is associated with low or normal serum parathyroid hormone concentrations (PTH). Approximately 50% of patients have mild or asymptomatic hypocalcemia; about 50% have paresthesias, carpopedal spasm, and seizures; about 10% have hypercalciuria with nephrocalcinosis or kidney stones; and more than 35% have ectopic and basal ganglia calcifications (summary by Nesbit et al., 2013). Thakker (2001) noted that patients with gain-of-function mutations in the CASR gene, resulting in generally asymptomatic hypocalcemia with hypercalciuria, have low-normal serum PTH concentrations and have often been diagnosed with hypoparathyroidism because of the insensitivity of earlier PTH assays. Because treatment with vitamin D to correct the hypocalcemia in these patients causes hypercalciuria, nephrocalcinosis, and renal impairment, these patients need to be distinguished from those with other forms of hypoparathyroidism (see 146200). Thakker (2001) suggested the designation 'autosomal dominant hypocalcemic hypercalciuria' for this CASR-related disorder. Genetic Heterogeneity of Autosomal Dominant Hypocalcemia Autosomal dominant hypocalcemia-2 (HYPOC2; 615361) is caused by mutation in the GNA11 gene (139313) on chromosome 19p13.
Autosomal dominant pseudohypoaldosteronism type 1
MedGen UID:
260623
Concept ID:
C1449842
Disease or Syndrome
Autosomal dominant pseudohypoaldosteronism type I is characterized by salt wasting resulting from renal unresponsiveness to mineralocorticoids. Patients may present with neonatal renal salt wasting with hyperkalaemic acidosis despite high aldosterone levels. These patients improve with age and usually become asymptomatic without treatment. Some adult patients with the disorder may have elevated aldosterone levels, but no history of clinical disease. This observation suggests that only those infants whose salt homeostasis is stressed by intercurrent illness and volume depletion develop clinically recognized PHA I (summary by Geller et al., 1998). Autosomal recessive pseudohypoaldosteronism type I (PHA1B; 264350), caused by mutation in any one of 3 genes encoding the epithelial sodium channel (ENaC), is a similar but more severe systemic disorder with persistence into adulthood.
Bartter disease type 3
MedGen UID:
335399
Concept ID:
C1846343
Disease or Syndrome
Bartter syndrome refers to a group of disorders that are unified by autosomal recessive transmission of impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and hypercalciuria. Clinical disease results from defective renal reabsorption of sodium chloride in the thick ascending limb (TAL) of the Henle loop, where 30% of filtered salt is normally reabsorbed (Simon et al., 1997). Patients with antenatal (or neonatal) forms of Bartter syndrome (e.g., BARTS1, 601678) typically present with premature birth associated with polyhydramnios and low birth weight and may develop life-threatening dehydration in the neonatal period. Patients with classic Bartter syndrome present later in life and may be sporadically asymptomatic or mildly symptomatic (summary by Simon et al., 1996 and Fremont and Chan, 2012). Genetic Heterogeneity of Bartter Syndrome Antenatal Bartter syndrome type 1 (601678) is caused by loss-of-function mutations in the butmetanide-sensitive Na-K-2Cl cotransporter NKCC2 (SLC12A1; 600839). Antenatal Bartter syndrome type 2 (241200) is caused by loss-of-function mutations in the ATP-sensitive potassium channel ROMK (KCNJ1; 600359). One form of neonatal Bartter syndrome with sensorineural deafness, Bartter syndrome type 4A (602522), is caused by mutation in the BSND gene (606412). Another form of neonatal Bartter syndrome with sensorineural deafness, Bartter syndrome type 4B (613090), is caused by simultaneous mutation in both the CLCNKA (602024) and CLCNKB (602023) genes. Also see autosomal dominant hypocalcemia-1 with Bartter syndrome (601198), which is sometimes referred to as Bartter syndrome type 5 (Fremont and Chan, 2012), caused by mutation in the CASR gene (601199). See Gitelman syndrome (GTLMN; 263800), which is often referred to as a mild variant of Bartter syndrome, caused by mutation in the thiazide-sensitive sodium-chloride cotransporter SLC12A3 (600968).
Bartter disease type 2
MedGen UID:
343428
Concept ID:
C1855849
Disease or Syndrome
Bartter syndrome refers to a group of disorders that are unified by autosomal recessive transmission of impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and hypercalciuria. Clinical disease results from defective renal reabsorption of sodium chloride in the thick ascending limb (TAL) of the Henle loop, where 30% of filtered salt is normally reabsorbed (Simon et al., 1997). Patients with antenatal forms of Bartter syndrome typically present with premature birth associated with polyhydramnios and low birth weight and may develop life-threatening dehydration in the neonatal period. Patients with classic Bartter syndrome (see BARTS3, 607364) present later in life and may be sporadically asymptomatic or mildly symptomatic (summary by Simon et al., 1996 and Fremont and Chan, 2012). For a discussion of genetic heterogeneity of Bartter syndrome, see 607364.
Bartter disease type 1
MedGen UID:
355727
Concept ID:
C1866495
Disease or Syndrome
Bartter syndrome refers to a group of disorders that are unified by autosomal recessive transmission of impaired salt reabsorption in the thick ascending loop of Henle with pronounced salt wasting, hypokalemic metabolic alkalosis, and hypercalciuria. Clinical disease results from defective renal reabsorption of sodium chloride in the thick ascending limb (TAL) of the Henle loop, where 30% of filtered salt is normally reabsorbed (Simon et al., 1997). Patients with antenatal forms of Bartter syndrome typically present with premature birth associated with polyhydramnios and low birth weight and may develop life-threatening dehydration in the neonatal period. Patients with classic Bartter syndrome (see BARTS3, 607364) present later in life and may be sporadically asymptomatic or mildly symptomatic (summary by Simon et al., 1996 and Fremont and Chan, 2012). For a discussion of genetic heterogeneity of Bartter syndrome, see 607364.
EAST syndrome
MedGen UID:
411243
Concept ID:
C2748572
Disease or Syndrome
Syndrome with characteristics of seizures, sensorineural deafness, ataxia, intellectual deficit, and electrolyte imbalance. It has been described in five patients from four families. The disease is caused by homozygous or compound heterozygous mutations in the KCNJ10 gene, encoding a potassium channel expressed in the brain, spinal cord, inner ear and kidneys. Transmission is autosomal recessive.
Corticosterone methyloxidase type 2 deficiency
MedGen UID:
483046
Concept ID:
C3463917
Disease or Syndrome
CMO type II deficiency is an autosomal recessive disorder caused by a defect in the final biochemical step of aldosterone biosynthesis, the 18-hydroxylation of 18-hydroxycorticosterone (18-OHB) to aldosterone. This enzymatic defect results in decreased aldosterone and salt-wasting associated with an increased serum ratio of 18-OHB to aldosterone. In CMO II deficiency, aldosterone can be low or normal, but at the expense of increased secretion of 18-OHB. These patients have a low ratio of corticosterone to 18-OHB (Portrat-Doyen et al., 1998). The CYP11B2 gene product also catalyzes an earlier step in aldosterone biosynthesis: the 18-hydroxylation of corticosterone to 18-OHB. A defect in that enzymatic step results in CMO type I deficiency (204300), an allelic disorder with an overlapping phenotype but distinct biochemical features. In CMO I deficiency, aldosterone is undetectable, whereas its immediate precursor, 18-OHB, is low or normal (Portrat-Doyen et al., 1998).
Bartter disease type 5
MedGen UID:
934787
Concept ID:
C4310820
Disease or Syndrome
Antenatal Bartter syndrome is a potentially life-threatening disease characterized by fetal polyuria, polyhydramnios, prematurity, and postnatal polyuria with persistent renal salt wasting. In transient antenatal Bartter syndrome-5, the onset of polyhydramnios and labor occur several weeks earlier than in other forms of Bartter syndrome. Polyuria lasts from a few days to 6 weeks, ending around 30 to 33 weeks of gestational age. Other features in the neonatal period include hypercalciuria, causing nephrocalcinosis in some cases, as well as hyponatremia, hypokalemia, and elevated renin and aldosterone; these subsequently resolve or normalize, although nephrocalcinosis may persist (Laghmani et al., 2016).
Hypokalemic tubulopathy and deafness
MedGen UID:
1785163
Concept ID:
C5543621
Disease or Syndrome
Hypokalemic tubulopathy and deafness (HKTD) is an autosomal recessive disorder characterized by hypokalemic tubulopathy with renal salt wasting, disturbed acid-base homeostasis, and sensorineural deafness (Schlingmann et al., 2021).
Pseudohypoaldosteronism, type IB2, autosomal recessive
MedGen UID:
1824028
Concept ID:
C5774255
Disease or Syndrome
Autosomal recessive pseudohypoaldosteronism type IB2 (PHA1B2) is characterized by renal salt wasting and high concentrations of sodium in sweat, stool, and saliva. The disorder involves multiple organ systems and is especially threatening in the neonatal period. Laboratory evaluation shows hyponatremia, hyperkalemia, and increased plasma renin activity with high serum aldosterone concentrations. Respiratory tract infections are common in affected children and may be mistaken for cystic fibrosis (CF; 219700). Aggressive salt replacement and control of hyperkalemia results in survival, and the disorder appears to become less severe with age (review by Scheinman et al., 1999).
Pseudohypoaldosteronism, type IB3, autosomal recessive
MedGen UID:
1824029
Concept ID:
C5774256
Disease or Syndrome
Autosomal recessive pseudohypoaldosteronism type IB3 (PHA1B3) is characterized by renal salt wasting and high concentrations of sodium in sweat, stool, and saliva. The disorder involves multiple organ systems and is especially threatening in the neonatal period. Laboratory evaluation shows hyponatremia, hyperkalemia, and increased plasma renin activity with high serum aldosterone concentrations. Respiratory tract infections are common in affected children and may be mistaken for cystic fibrosis (CF; 219700). Aggressive salt replacement and control of hyperkalemia results in survival, and the disorder appears to become less severe with age (review by Scheinman et al., 1999).

Professional guidelines

PubMed

Sezai A, Shiono M
J Cardiol 2016 Jan;67(1):15-21. Epub 2015 Oct 23 doi: 10.1016/j.jjcc.2015.09.011. PMID: 26476499
Waanders F, de Vries LV, van Goor H, Hillebrands JL, Laverman GD, Bakker SJ, Navis G
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Negro R
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Recent clinical studies

Etiology

Neuen BL, Oshima M, Agarwal R, Arnott C, Cherney DZ, Edwards R, Langkilde AM, Mahaffey KW, McGuire DK, Neal B, Perkovic V, Pong A, Sabatine MS, Raz I, Toyama T, Wanner C, Wheeler DC, Wiviott SD, Zinman B, Heerspink HJL
Circulation 2022 May 10;145(19):1460-1470. Epub 2022 Apr 8 doi: 10.1161/CIRCULATIONAHA.121.057736. PMID: 35394821
Puglisi S, Rossini A, Poli R, Dughera F, Pia A, Terzolo M, Reimondo G
Front Endocrinol (Lausanne) 2021;12:738848. Epub 2021 Oct 21 doi: 10.3389/fendo.2021.738848. PMID: 34745006Free PMC Article
Koch EAT, Nakhoul R, Nakhoul F, Nakhoul N
Int Urol Nephrol 2020 Sep;52(9):1705-1712. Epub 2020 Jul 13 doi: 10.1007/s11255-020-02545-4. PMID: 32661628
MacKenzie SM, van Kralingen JC, Davies E
Vitam Horm 2019;109:241-263. Epub 2018 Aug 7 doi: 10.1016/bs.vh.2018.07.001. PMID: 30678858
Rimoldi SF, Yuzefpolskaya M, Allemann Y, Messerli F
Prog Cardiovasc Dis 2009 Nov-Dec;52(3):249-59. doi: 10.1016/j.pcad.2009.10.002. PMID: 19917337

Diagnosis

Leal CRV, Costa LB, Ferreira GC, Ferreira AM, Reis FM, Simões E Silva AC
Pregnancy Hypertens 2022 Jun;28:15-20. Epub 2022 Feb 4 doi: 10.1016/j.preghy.2022.01.011. PMID: 35149272
Słomka A, Martucci G, Raffa GM, Malvindi PG, Żekanowska E, Lorusso R, Suwalski P, Kowalewski M
Adv Exp Med Biol 2021;1352:73-86. doi: 10.1007/978-3-030-85109-5_5. PMID: 35132595
Shimosawa T
Hypertens Res 2013 Aug;36(8):657-60. doi: 10.1038/hr.2013.69. PMID: 23912973
Rimoldi SF, Yuzefpolskaya M, Allemann Y, Messerli F
Prog Cardiovasc Dis 2009 Nov-Dec;52(3):249-59. doi: 10.1016/j.pcad.2009.10.002. PMID: 19917337
McGrath MF, de Bold ML, de Bold AJ
Trends Endocrinol Metab 2005 Dec;16(10):469-77. Epub 2005 Nov 2 doi: 10.1016/j.tem.2005.10.007. PMID: 16269246

Therapy

Neuen BL, Oshima M, Agarwal R, Arnott C, Cherney DZ, Edwards R, Langkilde AM, Mahaffey KW, McGuire DK, Neal B, Perkovic V, Pong A, Sabatine MS, Raz I, Toyama T, Wanner C, Wheeler DC, Wiviott SD, Zinman B, Heerspink HJL
Circulation 2022 May 10;145(19):1460-1470. Epub 2022 Apr 8 doi: 10.1161/CIRCULATIONAHA.121.057736. PMID: 35394821
Ravindran S, Munusamy S
J Cell Physiol 2022 Feb;237(2):1182-1205. Epub 2021 Oct 29 doi: 10.1002/jcp.30621. PMID: 34713897
Cherney DZ, Perkins BA, Soleymanlou N, Maione M, Lai V, Lee A, Fagan NM, Woerle HJ, Johansen OE, Broedl UC, von Eynatten M
Circulation 2014 Feb 4;129(5):587-97. Epub 2013 Dec 13 doi: 10.1161/CIRCULATIONAHA.113.005081. PMID: 24334175
Shimosawa T
Hypertens Res 2013 Aug;36(8):657-60. doi: 10.1038/hr.2013.69. PMID: 23912973
Russi E, Weigand K
Klin Wochenschr 1983 Jun 1;61(11):541-5. doi: 10.1007/BF01486843. PMID: 6348399

Prognosis

Słomka A, Martucci G, Raffa GM, Malvindi PG, Żekanowska E, Lorusso R, Suwalski P, Kowalewski M
Adv Exp Med Biol 2021;1352:73-86. doi: 10.1007/978-3-030-85109-5_5. PMID: 35132595
Zachariah JP, Wang Y, Newburger JW, deFerranti SD, Mitchell GF, Vasan RS
J Am Heart Assoc 2021 Mar 16;10(6):e018419. Epub 2021 Mar 1 doi: 10.1161/JAHA.120.018419. PMID: 33641350Free PMC Article
Kovesdy CP, Quarles LD
Am J Physiol Renal Physiol 2016 Jun 1;310(11):F1168-74. Epub 2016 Feb 10 doi: 10.1152/ajprenal.00606.2015. PMID: 26864938Free PMC Article
Patel SK, Velkoska E, Burrell LM
Clin Exp Pharmacol Physiol 2013 Aug;40(8):551-9. doi: 10.1111/1440-1681.12069. PMID: 23432153
Volpe M, Battistoni A, Chin D, Rubattu S, Tocci G
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Clinical prediction guides

Neuen BL, Oshima M, Agarwal R, Arnott C, Cherney DZ, Edwards R, Langkilde AM, Mahaffey KW, McGuire DK, Neal B, Perkovic V, Pong A, Sabatine MS, Raz I, Toyama T, Wanner C, Wheeler DC, Wiviott SD, Zinman B, Heerspink HJL
Circulation 2022 May 10;145(19):1460-1470. Epub 2022 Apr 8 doi: 10.1161/CIRCULATIONAHA.121.057736. PMID: 35394821
Zachariah JP, Wang Y, Newburger JW, deFerranti SD, Mitchell GF, Vasan RS
J Am Heart Assoc 2021 Mar 16;10(6):e018419. Epub 2021 Mar 1 doi: 10.1161/JAHA.120.018419. PMID: 33641350Free PMC Article
Cherney DZ, Perkins BA, Soleymanlou N, Maione M, Lai V, Lee A, Fagan NM, Woerle HJ, Johansen OE, Broedl UC, von Eynatten M
Circulation 2014 Feb 4;129(5):587-97. Epub 2013 Dec 13 doi: 10.1161/CIRCULATIONAHA.113.005081. PMID: 24334175
Volpe M, Battistoni A, Chin D, Rubattu S, Tocci G
Nutr Metab Cardiovasc Dis 2012 Apr;22(4):312-7. Epub 2012 Mar 6 doi: 10.1016/j.numecd.2011.12.006. PMID: 22402063
Hsueh WA, Baxter JD
Hypertension 1991 Apr;17(4):469-77. doi: 10.1161/01.hyp.17.4.469. PMID: 2013474

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