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Turkish Archives of Pediatrics Nov 2022Bartter syndrome and Gitelman syndrome are rare inherited tubulopathies characterized by hypokalemic, hypochloremic metabolic alkalosis. This study aimed to clarify the...
OBJECTIVE
Bartter syndrome and Gitelman syndrome are rare inherited tubulopathies characterized by hypokalemic, hypochloremic metabolic alkalosis. This study aimed to clarify the frequency of the phenotypic and genotypic subgroups, clinical features, long-term management, and prognosis of children diagnosed with Bartter syndrome and Gitelman syndrome in this study.
MATERIALS AND METHODS
Twenty-seven patients with Bartter syndrome and 6 patients with Gitelman syndrome, who were followed up between 2004 and 2020 in a single center, were included in the study.
RESULTS
The median age of diagnosis was 4 months in patients with Bartter syndrome and 174 months in patients with Gitelman syndrome. At the last follow-up, a total of 12 Bartter syndrome patients had chronic kidney disease with a mean 7.79 ± 4.73 years of age; 5 (18.5%) of these patients had chronic kidney disease stage 2, 5 (18.5%) had chronic kidney disease stage 3, and 2 (7.4%) had chronic kidney disease stage 5. Of the 5 patients with Bartter syndrome with chronic kidney disease stage 2, 2 had CLCNKB and 1 had SLC12A1 gene mutation. Also, CLCNKB mutation was detected in 2 of 5 patients with Bartter syndrome with chronic kidney disease stage 3. Finally, 2 patients with Bartter syndrome with chronic kidney disease stage 5 had BSND mutation in one and CLCNKB mutation in the other. Estimated glomerular filtration rates of all patients with Gitelman syndrome were normal at the last follow-up. There was no statistically significant association of development of chronic kidney disease with genetic mutation, nephrocalcinosis, prematurity, and hypokalemia.
CONCLUSION
Patients with Bartter syndrome and Gitelman syndrome may have a different clinical course due to the underlying genetic mutation. Bartter syndrome and Gitelman syndrome require lifelong treatment, and regular follow-up is important to prevent advanced-stage chronic kidney disease.
PubMed: 36314956
DOI: 10.5152/TurkArchPediatr.2022.22124 -
American Journal of Physiology. Renal... Mar 2023The Cl/[Formula: see text] exchanger pendrin in the kidney maintains acid-base balance and intravascular volume. Pendrin is upregulated in models associated with high...
The Cl/[Formula: see text] exchanger pendrin in the kidney maintains acid-base balance and intravascular volume. Pendrin is upregulated in models associated with high circulating aldosterone concentration, such as dietary NaCl restriction or an aldosterone infusion. However, it has not been established if pendrin is similarly regulated by aldosterone with a high-K diet because the effects of accompanying anions have not been considered. Here, we explored how pendrin is modulated by different dietary potassium salts. Wild-type (WT) and aldosterone synthase (AS) knockout (KO) mice were randomized to control, high-KHCO, or high-KCl diets. Dietary KCl and KHCO loading increased aldosterone in WT mice to the same extent but had opposite effects on pendrin abundance. KHCO loading increased pendrin protein and transcript abundance. Conversely, high-KCl diet feeding caused pendrin to decrease within 8 h of switching from the high-KHCO diet, coincident with an increase in plasma Cl and a decrease in [Formula: see text]. In contrast, switching the high-KCl diet to the high-KHCO diet caused pendrin to increase in WT mice. Experiments in AS KO mice revealed that aldosterone is necessary to optimally upregulate pendrin protein in response to the high-KHCO diet but not to increase pendrin mRNA. We conclude that pendrin is differentially regulated by different dietary potassium salts and that its regulation is prioritized by the dietary anion, providing a mechanism to prevent metabolic alkalosis with high-K base diets and safeguard against hyperchloremic acidosis with consumption of high-KCl diets. Regulation of the Cl/[Formula: see text] exchanger pendrin has been suggested to explain the aldosterone paradox. A high-K diet has been proposed to downregulate a pendrin-mediated K-sparing NaCl reabsorption pathway to maximize urinary K excretion. Here, we challenged the hypothesis, revealing that the accompanying anion, not K, drives pendrin expression. Pendrin is downregulated with a high-KCl diet, preventing acidosis, and upregulated with an alkaline-rich high-K diet, preventing metabolic alkalosis. Pendrin regulation is prioritized for acid-base balance.
Topics: Animals; Mice; Acidosis; Aldosterone; Alkalosis; Anion Transport Proteins; Bicarbonates; Diet; Potassium; Potassium, Dietary; Salts; Sodium Chloride; Sulfate Transporters
PubMed: 36656986
DOI: 10.1152/ajprenal.00128.2022 -
International Archives of Occupational... Oct 2021During the COVID-19 pandemic, healthcare professionals are recommended to use PPE to prevent the transmission of disease. Healthcare workers who use N95 FFR, which has...
OBJECTIVES
During the COVID-19 pandemic, healthcare professionals are recommended to use PPE to prevent the transmission of disease. Healthcare workers who use N95 FFR, which has an important place, experience complaints such as headache and dizziness. In this study, we plan to find the cause of these complaints and aim to clarify whether they are associated with the use of N95 mask.
METHOD
Healthcare workers first put on a surgical mask for at least 1 h and a maximum of 4 h, this process was then repeated on another day with the same workers wearing N95 masks. After removing the mask, capillary blood gases were taken and a questionnaire was given.
RESULTS
Thirty-four participants over the age of 18 were included in the study; 19 participants were female (56%) and 15 male (44%). The results of the capillary blood gas analysis after the use of surgical mask and N95 mask, respectively: pH: 7.43 ± 0.03; 7.48 ± 0.04 (p < 0.001); pCO: 37.33 ± 8.81; 28.46 ± 7.77 mmHg (p < 0.001); HCO: 24.92 ± 2.86; 23.73 ± 3.29 mmol/L (p = 0.131); Base excess (BE): 1.40 (- 3.90-3.10); - 2.68 (- 4.50-1.20) [median (Q1-Q3)] (p = 0.039); lactate: 1.74 ± 0.68; 1.91 ± 0.61 (p = 0314). Headache, attention deficit and difficulty in concentrating were significantly higher after using N95 mask.
CONCLUSION
Respiratory alkalosis and hypocarbia were detected after the use of N95. Acute respiratory alkalosis can cause headache, anxiety, tremor, muscle cramps. In this study, it was quantitatively shown that the participants' symptoms were due to respiratory alkalosis and hypocarbia.
Topics: Adult; Age Factors; Blood Gas Analysis; COVID-19; Dizziness; Female; Headache; Health Personnel; Hospitals, University; Humans; Hydrogen-Ion Concentration; Male; Masks; N95 Respirators; Pandemics; SARS-CoV-2; Sex Factors; Socioeconomic Factors
PubMed: 33646335
DOI: 10.1007/s00420-021-01665-3 -
PloS One 2017Although metabolic alkalosis is a common occurrence in intensive care units (ICUs), no study has evaluated its prevalence or outcomes in patients with severe sepsis or...
INTRODUCTION
Although metabolic alkalosis is a common occurrence in intensive care units (ICUs), no study has evaluated its prevalence or outcomes in patients with severe sepsis or septic shock.
METHODS
This is a retrospective cohort study of critically ill patients suffering from severe sepsis and septic shock admitted to the ICUs of Halmstad and Varberg County hospitals. From 910 patient records, 627 patients met the inclusion criteria. We investigated the relationship between metabolic alkalosis and mortality. Further, we studied the relationship between metabolic alkalosis and ICU length of stay (LOS).
RESULTS
Metabolic alkalosis was associated with decreased 30-day and 12-month mortalities. This effect was however lost when a multivariate analysis was conducted, correcting for age, gender, pH on admission, base excess (BE) on admission, Simplified Acute Physiology Score III (SAPS III) and acute kidney injury (AKI). We then analyzed for any dose-response effect between the severity of metabolic alkalosis and mortality and found no relationship. Bivariate analysis showed that metabolic alkalosis had a significant effect on the length of ICU stay. When adjusting for age, sex, pH at admission, BE at admission, SAPS III and AKI in a multivariate analysis, metabolic alkalosis significantly contributed to prolonged ICU length of stay. In two separate sensitivity analyses pure metabolic alkalosis and late metabolic alkalosis (time of onset >48 hours) were the only significant predictor of increased ICU length of stay.
CONCLUSION
Metabolic alkalosis did not have any effect on 30-day and 12-month mortalities after adjusting for age, sex, SAPS III-score, pH and BE on admission and AKI in a multivariate analysis. The presence of metabolic alkalosis was independently associated with an increased ICU length of stay.
Topics: Acute Kidney Injury; Aged; Alkalosis; Critical Illness; Female; Hospital Mortality; Humans; Hydrogen-Ion Concentration; Intensive Care Units; Length of Stay; Male; Middle Aged; Multivariate Analysis; Prevalence; Retrospective Studies; Sepsis; Severity of Illness Index; Shock, Septic; Treatment Outcome
PubMed: 28045915
DOI: 10.1371/journal.pone.0168563 -
Frontiers in Nutrition 2021
PubMed: 33842526
DOI: 10.3389/fnut.2021.669102 -
AJP Reports Jan 2024We describe a pregnant patient with severe compulsive water ingestion and vomiting that lead to metabolic alkalosis and preterm delivery. A 21-year-old patient was...
We describe a pregnant patient with severe compulsive water ingestion and vomiting that lead to metabolic alkalosis and preterm delivery. A 21-year-old patient was hospitalized multiple times throughout pregnancy for symptoms initially thought to be related to hyperemesis gravidarum. Overtime, it became apparent that the patient induced vomiting by rapidly drinking large volumes of water. At 32 weeks' gestation, rapid ingestion of water caused 3 days of vomiting with findings of hyponatremia, hypokalemia, hypochloremia, metabolic alkalosis, and compensatory respiratory acidosis. Fetal monitoring showed minimal variability and recurrent decelerations; subsequent biophysical profile score of 2/10 prompted urgent cesarean section. A male newborn was delivered and cord blood gases reflected neonatal metabolic alkalosis and electrolyte imbalances identical to those of the mother. Compensatory hypoventilation in both mother and fetus were treated with assisted ventilation. With saline administration and repletion of electrolytes, metabolic alkalosis resolved for both patients within days. Metabolic alkalosis was transplacentally acquired by the fetus. This case demonstrates the development of metabolic alkalosis in a pregnant woman caused by vomiting severe enough to prompt preterm delivery for nonreassuring fetal status. It also demonstrates fetal dependence on both placenta and mother to maintain physiologic acid-base and electrolyte balance.
PubMed: 38269119
DOI: 10.1055/s-0043-1778113 -
Indian Journal of Critical Care... Jan 2022Gopaldas JA. Revisiting Stewart's Approach toward Assessment of Unidentified or Complex Acid-Base Disorders. Indian J Crit Care Med 2022;26(1):5-6.
Gopaldas JA. Revisiting Stewart's Approach toward Assessment of Unidentified or Complex Acid-Base Disorders. Indian J Crit Care Med 2022;26(1):5-6.
PubMed: 35110833
DOI: 10.5005/jp-journals-10071-24099 -
Cureus Jan 2024Abiraterone acetate causes an adrenocorticotropic hormone (ACTH)-mediated mineralocorticoid excess. We present a 77-year-old man with prostate adenocarcinoma who...
Abiraterone acetate causes an adrenocorticotropic hormone (ACTH)-mediated mineralocorticoid excess. We present a 77-year-old man with prostate adenocarcinoma who developed signs and symptoms of mineralocorticoid excess while on abiraterone and discuss its pathophysiology and treatment options. The patient developed hypokalemia, metabolic alkalosis, and hypertension, indicative of increased mineralocorticoid activity, confirmed by elevated ACTH, corticosterone, and deoxycorticosterone levels. Abiraterone inhibits cytochrome P450c17 (CYP17A1), thus inhibiting testosterone and cortisol synthesis. Diminished cortisol synthesis, in turn, leads to excessive mineralocorticoid precursor production mediated by ACTH, leading to enhanced sodium absorption and potassium excretion. Abiraterone is often prescribed with low-dose prednisone to suppress ACTH; however, this strategy may not provide physiological glucocorticoid levels, resulting in ACTH-mediated mineralocorticoid excess in some patients. High-dose steroids or mineralocorticoid antagonists may activate mutant androgen receptors in prostate cancer tissue; therefore, amiloride is suggested for managing residual mineralocorticoid activity. This case highlights the importance of being vigilant for the signs and symptoms of mineralocorticoid excess in patients on abiraterone.
PubMed: 38318572
DOI: 10.7759/cureus.51757 -
Biochimica Et Biophysica Acta Dec 2014Soluble adenylyl cyclase (sAC) is poised to play multiple physiological roles as an acid/base (A/B) sensor in aquatic organisms. Many of these roles are probably similar... (Review)
Review
Soluble adenylyl cyclase (sAC) is poised to play multiple physiological roles as an acid/base (A/B) sensor in aquatic organisms. Many of these roles are probably similar to those in mammals; a striking example is the evolutionary conservation of a mechanism involving sAC, carbonic anhydrase and vacuolar H⁺-ATPase that acts as a sensor system and regulator of extracellular A/B in shark gills and mammalian epididymis and kidney. Additionally, the aquatic environment presents unique A/B and physiological challenges; therefore, sACs from aquatic organisms have likely evolved distinct kinetic properties as well as distinct physiological roles. sACs from aquatic organisms offer an excellent opportunity for studying the evolution of A/B sensing at both the molecular and whole organism levels. Moreover, this information could help understand and predict organismal responses to environmental stress based on mechanistic models.This article is part of a Special Issue entitled "The Role of Soluble Adenylyl Cyclase in Health and Disease," guest edited by J. Buck and L. R. Levin.
Topics: Acid-Base Equilibrium; Adenylyl Cyclases; Amino Acid Sequence; Animals; Bicarbonates; Biological Transport; Body Fluids; Carbon Dioxide; Humans; Hydrogen-Ion Concentration; Marine Biology; Models, Biological; Molecular Sequence Data; Photosynthesis; Sequence Homology, Amino Acid; Sharks
PubMed: 24971688
DOI: 10.1016/j.bbadis.2014.06.021 -
Intensive Care Medicine Experimental Apr 2022Acid-base status is important for understanding pathophysiology, making a diagnosis, planning effective treatment and monitoring progress of critically ill patients....
BACKGROUND/AIMS
Acid-base status is important for understanding pathophysiology, making a diagnosis, planning effective treatment and monitoring progress of critically ill patients. Manual calculations are cumbersome, easily result in wrong conclusions. We wanted to develop an automated assessment of acid-base status.
METHODS
A simplified adaptive MATLAB script processing all available theory to date was created, evaluated and used on blood gas analyses drawn immediately after admission to ICU. The script was compared to golden standard, calculating manually by two experienced ICU physicians.
RESULTS
Results from the script correlated completely with detailed manual calculations of randomly chosen 100 blood gas results and it was able to deliver complex data on cohort level with advanced graphics. The initial blood gas analyses from 8875 admissions constituted the cohort, of which 4111 (46.3%) were normal. Respiratory acidosis was the primary disturbance in 2753 (31.0%) and metabolic acidosis in 464 (5.2%). Respiratory alkalosis was the primary disturbance in 1501 (17.0%) and metabolic alkalosis in 46 (0.5%). Of the disturbances 74.7% were mixed with two and 2.1% with three simultaneous disturbances. Acidoses were less compensated compared to alkaloses.
CONCLUSIONS
Acid-base theories are developed on ideal models and not on critical care patients, they require inputs that might not be available, and therefore, estimations are needed. In our cohort, it was difficult to develop a working script based on Stewart, whereas Boston/Copenhagen worked better. Acidoses were more common and more deviated compared to alkaloses.
PubMed: 35377054
DOI: 10.1186/s40635-022-00437-8