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Cystic fibrosis (CF) is the most common life-threatening genetic disease in the United States and among people of European descent. Despite the widespread distribution of the cystic fibrosis transmembrane conductance regulator (CFTR) along kidney tubules, specific renal phenotypes attributable to CF have not been well documented. Recent studies have demonstrated the downregulation of the apical Cl-/HCO3 - exchanger pendrin (Slc26a4) in kidney B-intercalated cells of CF mouse models. These studies have shown that kidneys of both mice and humans with CF have an impaired ability to excrete excess HCO3 -, thus developing metabolic alkalosis when subjected to excess HCO3 - intake. The purpose of this minireview is to discuss the latest advances on the role of pendrin as a molecule with dual critical roles in acid base regulation and systemic vascular volume homeostasis, specifically in CF. Given the immense prevalence of vascular volume depletion, which is primarily precipitated via enhanced chloride loss through perspiration, we suggest that the dominant presentation of metabolic alkalosis in CF is due to the impaired function of pendrin, which plays a critical role in systemic vascular volume and acid base homeostasis.

BACKGROUND: Syndrome of apparent mineralocorticoid excess (AME) is characterized by excessive MR stimulation despite low levels of aldosterone. 11Beta-hydroxysteroid dehydrogenase-2 (11βDSH-2) inactivates cortisol to cortisone, preventing cortisol-induced MR activation. Genetic defects in 11βDSH-2 cause AME through accumulation of cortisol in the distal nephron, leading to MR activation induced hypertension, hypokalemia and metabolic alkalosis. Acquired AME can occur due to the ingestion of glycyrrhizic acid, found in licorice root, which inhibits 11βDSH-2 and has additional effects on cortisol homeostasis through inhibition of 11βDSH-1.
METHODS: We present a case of acquired AME with a hyperadrenergic symptoms induced by ingestion of Advanced Liver Support, a nutritional supplement produced by Advanced BioNutritionals(R), in a 65-year-old Caucasian female who presented with accelerated hypertension, hypokalemia, metabolic alkalosis and adrenergic symptoms. Cessation of the licorice-containing supplement resulted in complete resolution of the patient\'s hypertension, symptoms and abnormal lab values. To our knowledge this is the first reported case of AME from this supplement, and the first to describe accompanying hyperadrenergic symptoms.
CONCLUSIONS: Glycyrrhizic acid is increasingly being found in unregulated nutritional supplements and has the potential to induce a reversable syndrome of AME. Acquired AME should be suspected in individuals who present with hypertension along with hypokalemia, metabolic alkalosis and low plasma renin and serum aldosterone levels.

BACKGROUND: Sodium bicarbonate (NaHCO3) ingestion has been found to be ergogenic in high-intensity exercise that ranges from 1 to 10 min; however, limited studies have investigated high-intensity exercise beyond this duration.
OBJECTIVE: The present study aimed to determine the effect of NaHCO3 ingested using a carbohydrate hydrogel delivery system on 40 km time trial (TT) performance in trained male cyclists.
METHODS: Fourteen trained male cyclists ingested 0.3 g kg-1 BM NaHCO3 (Maurten AB, Sweden) to determine individualised peak alkalosis, which established time of ingestion prior to exercise. Participants completed a 40 km familiarisation TT, and two 40 km experimental TTs after ingestion of either NaHCO3 or placebo in a randomised, double-blind, crossover design.
RESULTS: NaHCO3 supplementation improved performance (mean improvement = 54.14 s ± 18.16 s; p = 0.002, g = 0.22) and increased blood buffering capacity prior to (HCO3- mean increase = 5.6 ± 0.2 mmol L-1, p < 0.001) and throughout exercise (f = 84.82, p < 0.001, pη2 = 0.87) compared to placebo. There were no differences in total gastrointestinal symptoms (GIS) between conditions either pre- (NaHCO3, 22 AU; Placebo, 44 AU; p = 0.088, r = 0.46) or post-exercise (NaHCO3, 76 AU; Placebo, 63 AU; p = 0.606, r = 0.14).
CONCLUSIONS: The present study suggests that ingesting NaHCO3 mini-tablets in a carbohydrate hydrogel can enhance 40 km TT performance in trained male cyclists, with minimal GIS. This ingestion strategy could therefore be considered by cyclists looking for a performance enhancing ergogenic aid.

BACKGROUND: Status epilepticus (SE) is a heterogeneous neurological emergency with significant variability in prognosis, influenced by underlying disease and pathophysiological context. Acid-base disturbances are common in critically ill patients, yet their distribution and impact in SE patients remain poorly understood.
METHODS: This was an observational cohort study including non-hypoxic SE patients with available blood gas analysis within the first 24 h of SE, treated at the University Hospital of Geneva, Switzerland between 2015 and 2023. Acid-base disturbances were classified using the Henderson-Hasselbalch equation, with prevalent metabolic alkalosis confirmed through the Stewart approach. Primary outcomes were in-hospital mortality, Glasgow Outcome Scale (GOS) at discharge, and return to premorbid neurologic function.
RESULTS: Among 540 SE patients, 365 were included. Half of patients exhibited acid-base disturbances within the initial 24 h of SE, with metabolic and respiratory acidosis being the most prevalent, though not prognostically significant. After correction for possible confounders, metabolic alkalosis (6%) was associated with increased in-hospital mortality (P = 0.011; OR = 4.87, 95% CI = 1.29-7.84), worse GOS (P = 0.012; OR = 3.18, 95% CI = 1.29-7.84), and reduced likelihood of returning to premorbid function (P = 0.017; OR = 3.30, CI95% = 1.24-8.80). Following the Stewart approach, 9% of patients had predominant metabolic alkalosis, associated with worse GOS (P = 0.005; OR:3.37, 95%CI = 1.45-7.82), and reduced chance of returning to baseline (P = 0.012; OR = 3.29, CI95% = 1.30-8.32). Metabolic alkalosis was related to hypoalbuminemia and lower serum potassium.
CONCLUSIONS: Metabolic alkalosis strongly predicts mortality and adverse functional outcome in SE patients. Prospective studies should assess whether early detection and correction of metabolic alkalosis and related electrolyte imbalances can improve SE prognosis.

BACKGROUND: An air embolism is a rare complication that occurs after air enters blood vessels, causing almost no to mild symptoms in patients. Although uncommon, air embolism can be deadly. Critical care professionals should know the warning signs of air embolism and be prepared to carry out the necessary therapeutic interventions. To reduce morbidity and death, this clinical condition must be identified early. Here we are presenting a case of pulmonary artery air embolism as a consequence of contrast agent injection in a chest computed tomography study.
METHODS: A 70-year-old male patient were presented with pulmonary artery air embolism as a consequence of contrast agent injection in a chest computed tomography study. The patient experienced worsening respiratory symptoms that necessitated oxygen therapy, which resulted in respiratory alkalosis with secondary metabolic alkalosis. Following removal of the BiLevel positive airway pressure, the patient was switched to a 2-L nasal cannula, and his breathing rate increased to 34 breaths/min. After 8.5 hours of monitoring the patient\'s vital signs, the nasal cannula was removed, and the patient began breathing room air on his own. His vital signs then stabilized and arterial blood gas parameters returned to normal. The patient\'s condition improved, and he was discharged from the hospital after 9 days. Due to a high level of cytomegalovirus, the discharge prescriptions included valganciclovir film-coated tablets (900 mg, oral BID every 12 hours for 30 days) and apixaban (5 mg BID). The patient was then monitored at the outpatient clinic.
CONCLUSIONS: Although rare, an air embolism can cause minor symptoms if it is small in volume or can be fatal if large. After contrast-enhanced radiological studies, physicians should be aware of any signs of respiratory distress or worsening of symptoms in their patients. Additionally, patients should be mindful of the potential complications associated with ventilation therapy.

BACKGROUND: Regional citrate anticoagulation (RCA) is recommended during continuous renal replacement therapy. Compared to systemic anticoagulation, RCA provides a longer filter lifespan with the risk of metabolic alkalosis and impaired calcium homeostasis. Surprisingly, most RCA protocols are designed for continuous veno-venous hemodialysis or hemodiafiltration. Effective protocols for continuous veno-venous hemofiltration (CVVH) are rare, although CVVH is a standard treatment for high-molecular-weight clearance. Therefore, we evaluated a new RCA protocol for postdilution CVVH.
METHODS: This is a monocentric prospective interventional study to evaluate a new RCA protocol for postdilution CVVH. We recruited surgical patients with stage III acute kidney injury who needed renal replacement therapy. We recorded dialysis and RCA data and hemodynamic and laboratory parameters during treatment sessions of 72 h. The primary endpoint was filter patency at 72 h. The major safety parameters were metabolic alkalosis and severe hypocalcemia at any time.
RESULTS: We included 38 patients who underwent 66 treatment sessions. The mean filter lifespan was 66 ± 12 h, and 44 of 66 (66%) filters were patent at 72 h. After censoring for non-CVVH-related cessation of treatment, 83% of all filters were patent at 72 h. The delivered dialysis dose was 28 ± 5 ml/kgBW/h. The serum levels of creatinine, urea and beta2-microglobulin decreased significantly from day 0 to day 3. Metabolic alkalosis occurred in one patient. An iCa++ below 1.0 mmol/L occurred in four patients. Citrate accumulation did not occur.
CONCLUSIONS: We describe a safe, effective, and easy-to-use RCA protocol for postdilution CVVH. This protocol provides a long and sustained filter lifespan without serious adverse effects. The risk of metabolic alkalosis and hypocalcemia is low. Using this protocol, a recommended dialysis dose can be safely administered with effective clearance of low- and middle-molecular-weight molecules.
BACKGROUND: The study was approved by the medical ethics committee of Heinrich-Heine University Duesseldorf (No. 2018-82KFogU). The trial was registered in the local study register of the university (No: 2018044660) on 07/04/2018 and was retrospectively registered at ClinicalTrials.gov (ClinicalTrials.gov Identifier: NCT03969966) on 31/05/2019.

Cystic fibrosis is a multisystem disease with extremely variable onset, symptoms and course. One of the onset modality but also a complication of the disease is the pseudo-Bartter syndrome, characterized by hyponatremia, hypochloremic dehydration and metabolic alkalosis in absence of any renal disease. This syndrome occurs more frequently in the first year of life and has a peak in the summer. In this article, we describe two cases of cystic fibrosis associated with pseudo-Bartter syndrome in childhood. Excluding every possible cause of metabolic alkalosis associated with hyponatremia was crucial for our diagnostic pathway, and the experience gained with the first case helped a lot with the second one.

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The base excess value (BE, mmol/L), not standard base excess (SBE), correctly calculated including pH, pCO2 (mmHg), sO2 (%) and cHb (g/dl) is a diagnostic tool for several in vivo events, e.g., mortality after multiple trauma or shock, acidosis, bleeding, clotting, artificial ventilation. In everyday clinical practice a few microlitres of blood (arterial, mixed venous or venous) are sufficient for optimal diagnostics of any metabolic acidosis or alkalosis.The same applies to a therapeutic tool-then referred to as potential base excess (BEpot)-for several in vitro assessments, e.g., solutions for infusion, sodium bicarbonate, blood products, packed red blood cells, plasma. Thus, BE or BEpot has been a parameter with exceptional clinical significance since 2007.