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Pseudohypoaldosteronism

A heterogeneous group of disorders characterized by renal electrolyte transport dysfunctions. Congenital forms are rare autosomal disorders characterized by neonatal hypertension, HYPERKALEMIA, increased RENIN activity and ALDOSTERONE concentration. The Type I features HYPERKALEMIA with sodium wasting; Type II, HYPERKALEMIA without sodium wasting. Pseudohypoaldosteronism can be the result of a defective renal electrolyte transport protein or acquired after KIDNEY TRANSPLANTATION.
Also Known As:
Pseudohypoaldosteronism Type 1, Autosomal Dominant; Pseudohypoaldosteronism Type 2; Familial Hypertensive Hyperkalemia; Familial Hypertensive Hyperkalemias; Gordon Hyperkalemia Hypertension Syndrome; Hyperkalemia, Familial Hypertensive; Hyperkalemia-Hypertension Syndrome, Gordon; Hyperkalemias, Familial Hypertensive; Hypertensive Hyperkalemias, Familial; Pseudohypoaldosteronism Type 1s; Pseudohypoaldosteronism Type 2s; Pseudohypoaldosteronisms; Pseudohypoaldosteronisms, Type I; Pseudohypoaldosteronisms, Type II; Syndrome, Gordon Hyperkalemia-Hypertension; Type 1, Pseudohypoaldosteronism; Type 1s, Pseudohypoaldosteronism; Type 2, Pseudohypoaldosteronism; Type 2s, Pseudohypoaldosteronism; Type I Pseudohypoaldosteronism; Type I Pseudohypoaldosteronisms; Type II Pseudohypoaldosteronism; Type II Pseudohypoaldosteronisms; Gordon Hyperkalemia-Hypertension Syndrome; Hyperpotassemia and Hypertension, Familial; Hypertensive Hyperkalemia, Familial; Pseudohypoaldosteronism Type 1; Pseudohypoaldosteronism Type 1, Autosomal Recessive; Pseudohypoaldosteronism, Type I; Pseudohypoaldosteronism, Type I, Autosomal Dominant; Pseudohypoaldosteronism, Type I, Autosomal Recessive; Pseudohypoaldosteronism, Type II
Networked: 297 relevant articles (1 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Hypertension (High Blood Pressure)
2. Hyperaldosteronism (Conn Syndrome)
3. Liddle Syndrome
4. Apparent Mineralocorticoid Excess Syndrome
5. Hypotension (Low Blood Pressure)

Experts

1. Uchida, Shinichi: 22 articles (11/2015 - 12/2005)
2. Rai, Tatemitsu: 17 articles (11/2015 - 10/2006)
3. Sasaki, Sei: 17 articles (11/2015 - 10/2006)
4. Sohara, Eisei: 15 articles (11/2015 - 10/2009)
5. Chiga, Motoko: 10 articles (11/2015 - 10/2006)
6. Lifton, Richard P: 10 articles (03/2009 - 01/2003)
7. Mori, Takayasu: 9 articles (11/2015 - 03/2013)
8. Zennaro, Maria-Christina: 9 articles (08/2013 - 06/2003)
9. Riepe, Felix G: 9 articles (05/2013 - 04/2003)
10. Kahle, Kristopher T: 9 articles (03/2009 - 01/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pseudohypoaldosteronism:
1. AldosteroneIBA
2. ReninIBA
3. Epithelial Sodium ChannelIBA
4. Lysine (L-Lysine)FDA Link
5. MineralocorticoidsIBA
6. Complementary DNA (cDNA)IBA
7. Phosphotransferases (Kinase)IBA
8. Mineralocorticoid Receptors (Mineralocorticoid Receptor)IBA
9. Protein Kinases (Protein Kinase)IBA
10. Proline (L-Proline)FDA Link
10/01/2012 - "Abnormal activation of the recently identified with-no-lysine kinase (WNK)-oxidative stress-responsive kinase 1 (OSR1)/STE20/SPS1-related proline/alanine-rich kinase (SPAK)-NaCl cotransporter (NCC) phosphorylation cascade results in the salt-sensitive hypertension of pseudohypoaldosteronism type II. Here, we report a study of renal WNK-OSR1/SPAK-NCC cascade activation in the db/db mouse model of hyperinsulinemic metabolic syndrome. "
01/01/2013 - "Stimulation of the OSR1 (Oxidative stress-responsive kinase-1)/SPAK [STE20 (sterile 20)/SPS1-related proline/alanine-rich kinase]-NCC (Na(+)-Cl(-) cotransporter) signaling cascade plays an important role in the WNK [With-No-Lysine (K)] kinase 4 D561A knock-in mouse model of pseudohypoaldosteronism type II (PHA II) characterized by salt-sensitive hypertension and hyperkalemia. "
01/01/2014 - "By analysing the pathogenesis of a hereditary hypertensive disease, PHAII (pseudohypoaldosteronism type II), we previously discovered that WNK (with-no-lysine kinase)-OSR1/SPAK (oxidative stress-responsive 1/Ste20-like proline/alanine-rich kinase) cascade regulates NCC (Na-Cl co-transporter) in the DCT (distal convoluted tubules) of the kidney. "
11/01/2013 - "WNKs (with-no-lysine kinases) are the causative genes of a hereditary hypertensive disease, PHAII (pseudohypoaldosteronism type II), and form a signal cascade with OSR1 (oxidative stress-responsive 1)/SPAK (STE20/SPS1-related proline/alanine-rich protein kinase) and Slc12a (solute carrier family 12) transporters. "

Therapies and Procedures

1. Fluid Therapy (Oral Rehydration Therapy)
2. Kidney Transplantation
3. Ileostomy