Virtually all of the dietary potassium intake is absorbed in the intestine, over 90% of which is excreted by the kidneys regarded as the most important organ of potassium excretion in the body. The renal excretion of potassium results primarily from the secretion of potassium by the principal cells in the aldosterone-sensitive distal nephron (ASDN), which is coupled to the reabsorption of Na⁺ by the epithelial Na⁺ channel (ENaC) located at the apical membrane of principal cells. When Na⁺ is transferred from the lumen into the cell by ENaC, the negativity in the lumen is relatively increased. K⁺ efflux, H⁺ efflux, and Cl^- influx are the 3 pathways that respond to Na⁺ influx, that is, all these 3 pathways are coupled to Na⁺ influx. In general, Na⁺ influx is equal to the sum of K⁺ efflux, H⁺ efflux, and Cl^- influx. Therefore, any alteration in Na⁺ influx, H⁺ efflux, or Cl^- influx can affect K⁺ efflux, thereby affecting the renal K⁺ excretion. Firstly, Na⁺ influx is affected by the expression level of ENaC, which is mainly regulated by the aldosterone-mineralocorticoid receptor (MR) pathway. ENaC gain-of-function mutations (Liddle syndrome, also known as pseudohyperaldosteronism), MR gain-of-function mutations (Geller syndrome), increased aldosterone levels (primary/secondary hyperaldosteronism), and increased cortisol (Cushing syndrome) or deoxycorticosterone (hypercortisolism) which also activate MR, can lead to up-regulation of ENaC expression, and increased Na⁺ reabsorption, K⁺ excretion, as well as H⁺ excretion, clinically manifested as hypertension, hypokalemia and alkalosis. Conversely, ENaC inactivating mutations (pseudohypoaldosteronism type 1b), MR inactivating mutations (pseudohypoaldosteronism type 1a), or decreased aldosterone levels (hypoaldosteronism) can cause decreased reabsorption of Na⁺ and decreased excretion of both K⁺ and H⁺, clinically manifested as hypotension, hyperkalemia, and acidosis. The ENaC inhibitors amiloride and Triamterene can cause manifestations resembling pseudohypoaldosteronism type 1b; MR antagonist spironolactone causes manifestations similar to pseudohypoaldosteronism type 1a. Secondly, Na⁺ influx is regulated by the distal delivery of water and sodium. Therefore, when loss-of-function mutations in Na⁺-K⁺-2Cl^- cotransporter (NKCC) expressed in the thick ascending limb of the loop and in Na⁺-Cl^- cotransporter (NCC) expressed in the distal convoluted tubule (Bartter syndrome and Gitelman syndrome, respectively) occur, the distal delivery of water and sodium increases, followed by an increase in the reabsorption of Na⁺ by ENaC at the collecting duct, as well as increased excretion of K⁺ and H⁺, clinically manifested as hypokalemia and alkalosis. Loop diuretics acting as NKCC inhibitors and thiazide diuretics acting as NCC inhibitors can cause manifestations resembling Bartter syndrome and Gitelman syndrome, respectively. Conversely, when the distal delivery of water and sodium is reduced (e.g., Gordon syndrome, also known as pseudohypoaldosteronism type 2), it is manifested as hypertension, hyperkalemia, and acidosis. Finally, when the distal delivery of non-chloride anions increases (e.g., proximal renal tubular acidosis and congenital chloride-losing diarrhea), the influx of Cl^- in the collecting duct decreases; or when the excretion of hydrogen ions by collecting duct intercalated cells is impaired (e.g., distal renal tubular acidosis), the efflux of H⁺ decreases. Both above conditions can lead to increased K⁺ secretion and hypokalemia. In this review, we focus on the regulatory mechanisms of renal potassium excretion and the corresponding diseases arising from dysregulation.
Humans ; Bartter Syndrome/metabolism* ; Pseudohypoaldosteronism/metabolism* ; Potassium/metabolism* ; Aldosterone/metabolism* ; Hypokalemia/metabolism* ; Gitelman Syndrome/metabolism* ; Hyperkalemia/metabolism* ; Clinical Relevance ; Epithelial Sodium Channels/metabolism* ; Kidney Tubules, Distal/metabolism* ; Sodium/metabolism* ; Hypertension ; Alkalosis/metabolism* ; Water/metabolism* ; Kidney/metabolism*

An 18-day-old male infant was admitted to the hospital due to recurrent hyperkalemia for more than 10 days. The neonate had milk refusal and dyspnea. The blood gas analysis revealed recurrent hyperkalemia, hyponatremia and metabolic acidosis. Adrenocortical hormone replacement therapy was ineffective. Additional tests showed a significant increase in aldosterone levels. Family whole exome sequencing revealed that the infant had compound heterozygous in the SCNNIA gene, inherited from both parents. The infant was diagnosed with neonatal systemic pseudohypoaldosteronism type I. The infant's electrolyte levels were stabilized through treatment with sodium polystyrene sulfonate and sodium supplement. The infant was discharged upon clinical recovery. This study provides a focused description of differential diagnosis of salt-losing syndrome in infants and introduces the multidisciplinary management of neonatal systemic pseudohypoaldosteronism type I.
Infant ; Infant, Newborn ; Humans ; Male ; Pseudohypoaldosteronism/genetics* ; Hyperkalemia/etiology* ; Hyponatremia/diagnosis* ; Diagnosis, Differential

Hyperkalemia was one of the complications after primary aldosteronism surgery. Hyperkalemia after primary aldosteronism surgery was uncommon in clinical practice, especially persistent and serious hyperkalemia was rare. This complication was not attached great importance in clinical work. A case about persistent and serious hyperkalemia after primary aldosteronism adrenal adenoma surgery was reported and the patient was followed-up for fourteen months in this study. This patient had a laparoscopic adrenalectomy due to primary aldosteronism. Hyperkalemia was detected one month after surgery of this patient, the highest level of plasma potassium was 7.0 mmol/L. The patient felt skin itchy, nausea, palpitation. Plasma aldosterone concentration fell to 2.12 ng/dL post-operation from 35.69 ng/dL pre-operation, zona glomerulosa insufficiency was confirmed by hormonal tests in this patient after surgery. And levels of 24 hours urinary potassium excretion declined. Decrease of aldosterone levels after surgery might be the cause of hyperkalemia. Hyperkalemia lasted for 14 months after surgery and kalemia-lowering drugs were needed. A systemic search with "primary aldosteronism", "hyperkalemia", "surgical treatment" was performed in PubMed and Wanfang Database for articles published between January 2009 and December 2019. Literature review indicated that the incidence of hyperkalemia after primary aldosteronism surgery was 6% to 29%. Most of them was mild to moderator hyperkalemia (plasma potassium 5.5 to 6.0 mmol/L) and transient. 19% to 33% in hyperkalemia patients was persistent hyperkalemia. Previous studies in the levels of plasma potassium reached the level as high as 7 mmol/L in our case were rare. Whether hypoaldosteronemia was the cause of hyperkalemia was not consistent in the published studies. Risk factors of hyperkalemia after primary aldosteronism surgery included kidney dysfunction, old age, long duration of hypertention. This paper aimed to improve doctors' aweareness of hyperkalemia complication after primary aldosteronism surgery. Plasma potassium should be monitored closely after primary aldosteronism surgery, especially in the patients with risk factors. Some patients could have persistent and serious hyperkalemia, and need medicine treatment.
Adrenalectomy/adverse effects* ; Aldosterone/therapeutic use* ; Humans ; Hyperaldosteronism/surgery* ; Hyperkalemia/surgery* ; Potassium/therapeutic use*

Objective: To explore the influencing factors for serum potassium >4.4 mmol/L in the morning of parathyroidectomy in hemodialysis patients with secondary hyperparathyroidism (SHPT). Methods: The clinical data of 72 patients with SHPT who received regular hemodialysis and underwent parathyroidectomy in Guangdong Provincial People's Hospital from January 2012 to December 2018 were analyzed retrospectively. There were 37 males and 35 females, aged from 25 to 69 years, and the dialysis timespan was from 0.5 to 11 years. The levels of parathyroid hormone, serum potassium and serum calcium before hemodialysis were examined one day before operation, and hemodialysis time and dewatering volume after hemodialysis without heparin were recorded, and also the level of serum potassium in the morning of parathyroidectomy was detected. The occurrences of hyperkalemia during and after operation were studied. The factors related to hyperkalemia in the morning of parathyroidectomy were evaluated by Pearson or Spearman correlation analysis, and the cut-off values of risk factors were calculated by receiver operating characteristic (ROC) curve. Results: Serum potassium >4.4 mmol/L in the morning of parathyroidectomy existed in 23 of 72 patients. Correlation analysis showed that serum potassium one day before operation ((4.93±0.56)mmol/L, r=0.656, P<0.001) and dehydration volume ((2.37±0.75)L, r=0.261, P=0.027) were positively correlated with serum potassium in the morning of parathyroidectomy((4.16±0.54)mmol/L). Serum potassium before hemodialysis one day before operation was a main predictor for serum potassium in the morning of parathyroidectomy (AUC=0.791, P<0.001). The cut-off value of serum potassium before hemodialysis one day before operation was 5.0 mmol/L. Conclusion: Serum potassium before hemodialysis one day before operation in patients with SHPT can predict serum potassium in the morning of parathyroidectomy, offering imformation for the safety of operation.
Calcium ; Female ; Humans ; Hyperkalemia/etiology* ; Hyperparathyroidism, Secondary/surgery* ; Male ; Parathyroid Hormone ; Parathyroidectomy ; Renal Dialysis ; Retrospective Studies

Pseudohypoaldosteronism (PHA) in infants is manifested by presence of hyperkalemia, hyponatremia, and metabolic acidosis. At initial stages, PAH is generally suspected as congenital adrenal hyperplasia. Transient PHA has been reported in infants with urinary tract infection and urinary tract malformation. We report a case of 5-month-old infant with failure to thrive and finally diagnosed with transient PHA due to urinary tract infection with vesicoureteral reflux.
Acidosis ; Adrenal Hyperplasia, Congenital ; Failure to Thrive* ; Humans ; Hyperkalemia ; Hyponatremia ; Infant* ; Pseudohypoaldosteronism* ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

BACKGROUND: Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy that anesthesiologists can encounter in the operation room, and patients with DMD are susceptible to complications such as rhabdomyolysis, hyperkalemic cardiac arrest, and hyperthermia during the perioperative period. Acute onset of hyperkalemic cardiac arrest is a crisis because of the difficulty in achieving satisfactory resuscitation owing to the sustained hyperkalemia accompanied by rhabdomyolysis. CASE: We here report a case of a 13-year-old boy who had multiple leg fractures and other trauma after a car accident and who had suffered from acute hyperkalemic cardiac arrest. He was refractory to cardiopulmonary resuscitation and showed sustained hyperkalemia. With extracorporeal membrane oxygenation and in-line hemofiltration, he recovered from repeated cardiac arrest and hyperkalemia. CONCLUSIONS: Combining ECMO and in-line hemofiltration might be a safe and effective technique for refractory hyperkalemic cardiac arrest and rhabdomyolysis in patients with DMD.
Adolescent ; Cardiopulmonary Resuscitation ; Extracorporeal Membrane Oxygenation ; Fever ; Heart Arrest ; Hemofiltration ; Humans ; Hyperkalemia ; Leg ; Male ; Muscular Dystrophies ; Muscular Dystrophy, Duchenne ; Orthopedics ; Perioperative Period ; Resuscitation ; Rhabdomyolysis

PURPOSE: Whereas continuous renal replacement therapy (CRRT) has been utilized during liver transplantation (LT), there was a lack of evidence to support this practice. We investigated the adverse events at the perioperative periods in recipients of LT who received preoperative CRRT without intraoperative CRRT. METHODS: We retrospectively reviewed medical records of adult patients (age ≥ 18 years) who received LT between December 2009 and May 2015. Perioperative data were collected from the recipients, who received preoperative CRRT until immediately before LT, because of refractory renal dysfunction. RESULTS: Of 706 recipients, 42 recipients received preoperative CRRT. The mean (standard deviation) Model for end-stage liver disease score were 49.6 (13.4). Twenty-six point two percent (26.2%) of recipients experienced the serum potassium > 4.5 mEq/L before reperfusion and treated with regular insulin. Thirty-eight point one percent (38.1%) of recipients were managed with sodium bicarbonate because of acidosis (base excess <−10 mEq/L throughout LT). All patients finished their operations without medically uncontrolled complications such as severe hyperkalemia (serum potassium > 5.5 mEq/L), refractory acidosis, or critical arrhythmias. Mortality was 19% at 30 day and 33.3% at 1 year. CONCLUSION: Although intraoperative CRRT was not used in recipients with severe preoperative renal dysfunction, LT was safely performed. Our experience raises a question about the need for intraoperative CRRT.
Acidosis ; Adult ; Arrhythmias, Cardiac ; Humans ; Hyperkalemia ; Insulin ; Liver Diseases ; Liver Transplantation ; Liver* ; Medical Records ; Mortality ; Perioperative Period ; Potassium ; Renal Replacement Therapy ; Reperfusion ; Retrospective Studies ; Sodium Bicarbonate ; Transplant Recipients*

PURPOSE: The purpose of this study was to identify the causes, symptoms, and complications of hypoproteinemia to prevent hypoproteinemia and provide appropriate treatment to children with atopic dermatitis. METHODS: Children diagnosed with atopic dermatitis with hypoproteinemia and/or hypoalbuminemia were retrospectively reviewed. The patients’ medical records, including family history, weight, symptoms, treatment, complications, and laboratory test results for allergies and skin cultures, were examined. RESULTS: Twenty-six patients (24 boys) were enrolled. Seven cases had growth retardation; 7, keratoconjunctivitis; 6, aural discharges; 5, eczema herpeticum; 4, gastrointestinal tract symptoms; and 2, developmental delays. In 21 cases, topical steroids were not used. According to the blood test results, the median values of each parameter were elevated: total IgE, 1,864 U/mL; egg white-specific IgE, 76.5 kU(A)/L; milk IgE, 20.5 kU(A)/L; peanut IgE, 30 kU(A)/L; eosinophil count, 5,810/μL; eosinophil cationic protein, 93.45 μg/L; and platelet count, 666.5×10³/μL. Serum albumin and total protein levels decreased to 2.7 g/dL and 4.25 g/dL, respectively. Regarding electrolyte abnormality, 10 patients had hyponatremia, and 12, hyperkalemia. Systemic antibiotics were used to treat all cases, and an antiviral agent was used in 12 patients. Electrolyte correction was performed in 8 patients. CONCLUSION: Hypoproteinemia accompanying atopic dermatitis is common in infants younger than 1 year and may occur because of topical steroid treatment continuously being declined or because of eczema herpeticum. It may be accompanied by growth retardation, keratoconjunctivitis, aural discharge, and eczema herpeticum and can be managed through skin care and topical steroid application without intravenous albumin infusion.
Anti-Bacterial Agents ; Arachis ; Child ; Dermatitis, Atopic* ; Eosinophil Cationic Protein ; Eosinophils ; Gastrointestinal Tract ; Hematologic Tests ; Humans ; Hyperkalemia ; Hypersensitivity ; Hypoalbuminemia ; Hyponatremia ; Hypoproteinemia* ; Immunoglobulin E ; Infant ; Kaposi Varicelliform Eruption ; Keratoconjunctivitis ; Medical Records ; Milk ; Ovum ; Platelet Count ; Retrospective Studies ; Serum Albumin ; Skin ; Skin Care ; Steroids

Since the implementation of the model for end-stage liver disease (MELD) scoring system in 2002, the liver transplantation (LT) society has observed a substantial increase in the number of recipients with renal dysfunction. Intraoperative renal replacement therapy (ioRRT) has emerged as one of the solutions available to manage high-MELD score recipients; however, its usefulness has not yet been proven. To date, we have experienced five cases of simultaneous liver and kidney transplantation (SLKT). Recipients of SLKT tend to have a lower pre-transplant kidney function and the longer operation time mandates a larger amount of fluid than LT alone. Hence, anesthetic care is more prone to be challenged by hyperkalemia, metabolic acidosis, and volume overload, making ioRRT a theoretically valuable intervention. However, in all five cases, recipients were managed without ioRRT, resulting in excellent graft and patient survival. As such, in this case series, we discuss current issues about ioRRT and SLKT.
Acidosis ; Humans ; Hyperkalemia ; Kidney Transplantation* ; Kidney* ; Liver Diseases ; Liver Transplantation ; Liver* ; Renal Replacement Therapy* ; Transplants

The ST segment corresponds to the plateau phase of ventricular repolarization, i.e., phase 2 of the action potential. Heightened awareness of the characteristic patterns of ST segment changes is vital to quickly identifying life-threatening disorders. The differential diagnosis of ST segment elevation includes four major processes: ST segment elevation myocardial infarction (STEMI); early repolarization; pericarditis; and ST elevation secondary to an abnormality of the QRS complex (left bundle branch block, left ventricular hypertrophy, or preexcitation). Other processes that may be associated with ST elevation include hyperkalemia, pulmonary embolism, and Brugada syndrome. Two particular patterns of ST segment depression reflect STEMI rather than non-ST-segment elevation acute coronary syndrome: ST segment depression that is reciprocal to a subtle and sometimes overlooked ST-segment elevation, and ST segment depression that is maximal in leads V₁-V₃, suggesting true posterior infarction. The clinical setting and specific electrocardiographic criteria often allow identification of the cause.
Action Potentials ; Acute Coronary Syndrome ; Brugada Syndrome ; Bundle-Branch Block ; Depression ; Diagnosis, Differential ; Electrocardiography ; Hyperkalemia ; Hypertrophy, Left Ventricular ; Infarction ; Myocardial Infarction ; Pericarditis ; Pulmonary Embolism