Primary hyperaldosteronism differential diagnosis: Difference between revisions

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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Headache
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Diagnoses
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nausea
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Vomiting
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Nausea and Vomiting
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Palpitations
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Palpitations
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Shortness of breath
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Shortness of breath
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* Chronic headaches
* Chronic headaches
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* Normal renal function tests
* Normal [[renal function tests]]
* Normal liver function tests
* Normal [[liver function tests]]
* Metabolic alkalosis (pH > 7.45)
* [[Metabolic alkalosis]] (pH > 7.45)
* Hyperkalemia  
* [[Hyperkalemia]]
* Plasma renin-aldosterone ratio <10
* [[Plasma]] [[renin]]-[[aldosterone]] ratio <10
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Coarctation of aorta]]
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Coarctation of aorta]]
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*Young patients (neonates) may have history of:
*Young patients ([[neonates]]) may have history of:
** Failure to thrive
** [[Failure to thrive]]
** Poor feeding
** Poor feeding
** Lethargy
** Lethargy
** Turner's syndrome
** [[Turner syndrome|Turner's syndrome]]
** Familial predisposition
** Familial predisposition
** Ventricular septal defects
** [[Ventricular septal defects]]
*Adults may have a history of:
*Adults may have a history of:
** Claudication
** [[Claudication]]
** Epistaxis
** [[Epistaxis]]
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* Bicuspid aortic valves
* [[Bicuspid aortic valves]]
* Notching of ribs
* Notching of [[ribs]]
* Metabolic alkalosis (pH > 7.45)
* [[Metabolic alkalosis]] (pH > 7.45)
* Hyperkalemia  
* [[Hyperkalemia]]
* Plasma renin-aldosterone ratio <10
* [[Plasma]] [[renin]]-[[aldosterone]] ratio <10
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| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |11-beta hydroxylase deficiency
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[11β-hydroxylase deficiency|11-beta hydroxylase deficiency]]
| style="padding: 5px 5px; background: #F5F5F5;" | ✔ (Hypertensive crisis due to increased 11-deoxycorticosterone-11-DOC)
| style="padding: 5px 5px; background: #F5F5F5;" | ✔ ([[Hypertensive crisis]] due to increased [[11-deoxycorticosterone]]-11-DOC)
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* Females:
** [[Clitoral body|Clitoral]] enlargement
** [[Labioscrotal folds|Labioscrotal]] fusion
* Males:
** [[Penis|Penile]] enlargement
* (If not diagnosed at birth, may present as premature [[adrenarche]], developing body odor with [[Axillary hair|axillary]] and [[pubic hair]] development)
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* Hypokalemia
* Increased 11-DOC levels
* Increased androgens
* Low [[urinary]] [[aldosterone]] level
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| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[17 alpha-hydroxylase deficiency|17-alpha hydroxylase deficiency]]
| style="padding: 5px 5px; background: #F5F5F5;" | ✔
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* Females:
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** Clitoral enlargement and labioscrotal fusion
* [[Phenotypically]] females at birth
* Lack of [[pubertal]] development in females
* Incompletely developed external [[genitalia]] in males
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* [[Focal neurologic signs|Focal neurological signs]] present ([[motor neuron disease]])
* Increased [[serum]] [[mineralocorticoids]]
* [[Extrapyramidal|Extrapyramidal signs]]  present (familial [[Frontotemporal lobar degenerations|frontotemporal lobar degeneration]])
* Decreased [[androgen]] levels
 
* [[Hypokalemia]]
* Onset in young age
* Low [[urinary]] [[aldosterone]] level
 
|-
* Strong [[familial]] association
|'''[[Uremia]]'''
 
| -✔
* Focal [[atrophy]] of [[Frontal lobe|frontal]] and [[temporal lobes]]
|✔
* Knife-edge [[atrophy]] noted on [[Magnetic resonance imaging|MRI]]
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* [[Personality changes|Personality]] and behavioral changes
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* Language impairment
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* Two major variants:
* Patients have [[chronic kidney disease]] and maybe on [[dialysis]]
** Progressive nonfluent [[aphasia]]
* Features of uremic neuropathy:
** [[Semantic Dementia|Semantic]] variant
** [[Autonomic nervous system|Autonomic]] features with postural [[hypotension]],
* [[Progressive supranuclear palsy]]
** Impaired [[sweating]]
** [[Diarrhea]]
** Impotence
** [[Paraesthesia]]
** Delayed [[Deep tendon reflex|deep tendon reflexes]]
** [[Muscle wasting]]
* [[Encephalopathy]]
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* [[Pick bodies]]
* Increased [[blood urea nitrogen]] ([[Blood urea nitrogen|BUN]]) and [[creatinine]] ([[Cr]])
* [[Corticobasal degeneration]]
* [[Hyperkalemia]]
* [[Ubiquitination|Ubiquitinated]] inclusions
* Decreased [[serum]] [[Vitamin D3|vitamin 1,25 dihydroxy vitamin D3]] level
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| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[Vascular dementia]]
|[[Liddle's syndrome|'''Liddle's syndrome''']]  
| style="padding: 5px 5px; background: #F5F5F5;" | + (Dysexecutive syndrome)
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* [[Focal neurologic signs|Focal neurological signs]] present typically
* Mild [[extrapyramidal]] signs
* [[Depression]]
* Mild [[Motor disorders|motor signs]] in subcortical [[vascular dementia]]
* Slowed processing speed
* Presentation might be similar to Alzheimer's disease
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* Stepwise progression and focal neurologic signs (also known as [[multi-infarct dementia]] or poststroke [[dementia]])
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* Multifocal and/or diffuse lesions (lacunes and microinfarcts)
* The following [[brain]] areas may be involved:
** [[Subcortical|Subcortical area]]
** [[Thalamus]]
** Frontobasal[[limbic system]]
** [[White matter]] lesions
** [[Hippocampal sclerosis]]
** Diffuse post-ischemic lesions
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Revision as of 15:42, 16 October 2017

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Overview

Primary hyperaldosteronism must be differentiated from other diseases that cause hypertension and hypokalemia such as renal artery stenosis, cushing's syndrome, congenital adrenal hyperplasia, Liddle's syndrome, diuretic use, licorice ingestion and renin-secreting tumors.

Differentiating Primary Hyperaldosteronism from other Diseases

Primary hyperaldosteronism (PA) should be differentiated from other diseases causing hypertension and hypokalemia for example:[1][1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]

 
 
 
 
 
 
 
 
Hypertension and Hypokalemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Plasma renin activity
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal or High (Plasma Renin/Aldosterone ratio <10
 
 
 
 
 
 
 
 
 
 
 
Suppressed (Plasma Renin/Aldosterone ratio >20
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
*Renin-secreting tumors
*Diuretic use
*Renovascular hypertension
*Coarctation of aorta
*Malignant phase hypertension
 
 
 
 
 
 
 
 
 
 
 
Urinary aldosterone
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Elevated
 
Normal
 
 
Low
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Conn's syndrome (Primary aldosteronism)
 
Profound K+ depletion
 
 
• 17 alpha hydroxylase deficiency
• 11 beta hydroxylase deficiency
• Liddle's syndrome
• Licorice ingestion
• Deoxycortisone producing tumor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Add Mineralocrticoid antagonist for 8 weeks
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)
• Licorice ingestion
•Glucocorticoid resistance
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Liddle's syndrome)
Differential Diagnoses Clinical features History Findings Laboratory Findings
Diagnoses Hypertension Nausea and Vomiting Palpitations Shortness of breath Diminished pulses Fatigue Constipation Visual Abnormalities Pruritis Polyuria Ambiguous genitalia
Renin-Secreting tumors ✔

(Due to hypertension)

✔ ✔ ✔ - - - - - -
  • Drug-resistant hypertension
  • Chronic headaches
Coarctation of aorta ✔ ✔ ✔ ✔ ✔ ✔ ✔ - - -
11-beta hydroxylase deficiency ✔ (Hypertensive crisis due to increased 11-deoxycorticosterone-11-DOC) ✔ ✔ - - ✔ - - - - ✔
17-alpha hydroxylase deficiency ✔ ✔ ✔ - - - - - - - ✔
Uremia -✔ ✔ ✔ ✔ - ✔ ✔ - ✔ - -
Liddle's syndrome

References

  1. ↑ 1.0 1.1 Wada N, Jin S, Hui SP, Yanagisawa K, Kurosawa T, Chiba H (2014). "[Differential diagnosis of primary aldosteronism by measurement of hybrid steroids using mass spectrometry]". Rinsho Byori (in Japanese). 62 (3): 276–82. PMID 24800505.
  2. ↑ Nielsen ML, Pareek M, Andersen I (2012). "[Liquorice-induced hypertension and hypokalaemia]". Ugeskr. Laeg. (in Danish). 174 (15): 1024–5. PMID 22487411.
  3. ↑ Chow KM, Ma RC, Szeto CC, Li PK (2012). "Polycystic kidney disease presenting with hypertension and hypokalemia". Am. J. Kidney Dis. 59 (2): 270–2. doi:10.1053/j.ajkd.2011.08.020. PMID 21962616.
  4. ↑ Sarafidis PA, Georgianos PI, Germanidis G, Giavroglou C, Nikolaidis P, Lasaridis AN, Madias NE (2012). "Hypertension and symptomatic hypokalemia in a patient with simultaneous unilateral stenoses of intrarenal arteries and mesangioproliferative glomerulonephritis". Am. J. Kidney Dis. 59 (3): 434–8. doi:10.1053/j.ajkd.2011.11.001. PMID 22154539.
  5. ↑ Khosla N, Hogan D (2006). "Mineralocorticoid hypertension and hypokalemia". Semin. Nephrol. 26 (6): 434–40. doi:10.1016/j.semnephrol.2006.10.004. PMID 17275580.
  6. ↑ Weiner ID (2013). "Endocrine and hypertensive disorders of potassium regulation: primary aldosteronism". Semin. Nephrol. 33 (3): 265–76. doi:10.1016/j.semnephrol.2013.04.007. PMC 3748390. PMID 23953804.
  7. ↑ Martell-Claros N, Abad-Cardiel M, Alvarez-Alvarez B, García-Donaire JA, Pérez CF (2015). "Primary aldosteronism and its various clinical scenarios". J. Hypertens. 33 (6): 1226–32. doi:10.1097/HJH.0000000000000546. PMID 25715092.
  8. ↑ Franse LV, Pahor M, Di Bari M, Somes GW, Cushman WC, Applegate WB (2000). "Hypokalemia associated with diuretic use and cardiovascular events in the Systolic Hypertension in the Elderly Program". Hypertension. 35 (5): 1025–30. PMID 10818057.
  9. ↑ Rossi E, Farnetti E, Nicoli D, Sazzini M, Perazzoli F, Regolisti G, Grasselli C, Santi R, Negro A, Mazzeo V, Mantero F, Luiselli D, Casali B (2011). "A clinical phenotype mimicking essential hypertension in a newly discovered family with Liddle's syndrome". Am. J. Hypertens. 24 (8): 930–5. doi:10.1038/ajh.2011.76. PMID 21525970.
  10. ↑ Ruecker B, Lang-Muritano M, Spanaus K, Welzel M, l'Allemand D, Phan-Hug F, Katschnig C, Konrad D, Holterhus PM, Schoenle EJ (2015). "The Aldosterone/Renin Ratio as a Diagnostic Tool for the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants". Horm Res Paediatr. 84 (1): 43–8. doi:10.1159/000381852. PMID 25968592.
  11. ↑ Ardhanari S, Kannuswamy R, Chaudhary K, Lockette W, Whaley-Connell A (2015). "Mineralocorticoid and apparent mineralocorticoid syndromes of secondary hypertension". Adv Chronic Kidney Dis. 22 (3): 185–95. doi:10.1053/j.ackd.2015.03.002. PMID 25908467.
  12. ↑ Iglesias P, Tajada P, Martínez I, Díez JJ (2009). "[Salt-wasting congenital adrenal hyperplasia associated to hyperreninemic hyperaldosteronism]". Med Clin (Barc) (in Spanish; Castilian). 132 (2): 80–1. doi:10.1016/j.medcli.2008.09.002. PMID 19174076.
  13. ↑ Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M (1988). "Primary aldosteronism in childhood due to primary adrenal hyperplasia". Tohoku J. Exp. Med. 155 (1): 57–70. PMID 3413779.
  14. ↑ Hassan-Smith Z, Stewart PM (2011). "Inherited forms of mineralocorticoid hypertension". Curr Opin Endocrinol Diabetes Obes. 18 (3): 177–85. doi:10.1097/MED.0b013e3283469444. PMID 21494136.
  15. ↑ Bartter FC, Henkin RI, Bryan GT (1968). "Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia". J. Clin. Invest. 47 (8): 1742–52. doi:10.1172/JCI105864. PMC 297334. PMID 4299011.

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