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Cureus Feb 2021Milk-alkali syndrome or calcium-alkali syndrome (CAS) is the triad of hypercalcemia, metabolic alkalosis and renal impairment. It is often related to ingestion of high... (Review)
Review
Milk-alkali syndrome or calcium-alkali syndrome (CAS) is the triad of hypercalcemia, metabolic alkalosis and renal impairment. It is often related to ingestion of high amounts of calcium carbonate, which was used historically for the treatment of peptic ulcer disease. The incidence of the syndrome decreased dramatically after the introduction of newer peptic ulcer medications such as proton pump inhibitors and histamine blocking agents. However, a resurgence was seen in the late 1980s with the wide use of over-the-counter calcium supplements, mainly by females for osteoporosis prophylaxis. The modern version of the syndrome continues to evolve along with medical management. This review focuses on the historical context of CAS, pathogenesis, resurgence of the condition with variable presentations, and contemporary management.
PubMed: 33732556
DOI: 10.7759/cureus.13291 -
Kidney Diseases (Basel, Switzerland) Dec 2017Kidneys play a pivotal role in the maintenance and regulation of acid-base and electrolyte homeostasis, which is the prerequisite for numerous metabolic processes and... (Review)
Review
Kidneys play a pivotal role in the maintenance and regulation of acid-base and electrolyte homeostasis, which is the prerequisite for numerous metabolic processes and organ functions in the human body. Chronic kidney diseases compromise the regulatory functions, resulting in alterations in electrolyte and acid-base balance that can be life-threatening. In this review, we discuss the renal regulations of electrolyte and acid-base balance and several common disorders including metabolic acidosis, alkalosis, dysnatremia, dyskalemia, and dysmagnesemia. Common disorders in chronic kidney disease are also discussed. The most recent and relevant advances on pathophysiology, clinical characteristics, diagnosis, and management of these conditions have been incorporated.
PubMed: 29344508
DOI: 10.1159/000479968 -
American Journal of Physiology. Renal... Feb 2016Pendrin is a Na(+)-independent Cl(-)/HCO3(-) exchanger found in the apical regions of type B and non-A, non-B intercalated cells within the aldosterone-sensitive region... (Review)
Review
Pendrin is a Na(+)-independent Cl(-)/HCO3(-) exchanger found in the apical regions of type B and non-A, non-B intercalated cells within the aldosterone-sensitive region of the nephron, i.e., the distal convoluted tubule (DCT), the connecting tubule (CNT), and the cortical collecting duct (CCD). Type B intercalated cells mediate Cl(-) absorption and HCO3(-) secretion primarily through pendrin-mediated Cl(-)/HCO3(-) exchange. This exchanger is upregulated with angiotensin II administration and in models of metabolic alkalosis, such as following administration of aldosterone or NaHCO3. In the absence of pendrin-mediated HCO3(-) secretion, an enhanced alkalosis is observed following aldosterone or NaHCO3 administration. However, probably of more significance is the role of pendrin in the pressor response to aldosterone. Pendrin mediates Cl(-) absorption and modulates aldosterone-induced Na(+) absorption mediated by the epithelial Na channel (ENaC). Pendrin changes ENaC activity by changing both channel open probability (Po) and surface density (N), at least partly by altering luminal HCO3(-) and ATP concentration. Thus aldosterone and angiotensin II stimulate pendrin expression and function, which stimulates ENaC activity, thereby contributing to the pressor response of these hormones. However, pendrin may modulate blood pressure partly through its extrarenal effects. For example, pendrin is expressed in the adrenal medulla, where it modulates catecholamine release. The increase in catecholamine release observed with pendrin gene ablation likely contributes to the increment in vascular contractile force observed in the pendrin null mouse. This review summarizes the signaling mechanisms that regulate pendrin abundance and function as well as the contribution of pendrin to distal nephron function.
Topics: Adenosine Triphosphate; Aldosterone; Angiotensin II; Animals; Bicarbonates; Blood Pressure; Epithelial Sodium Channels; Gene Expression Regulation; Humans; Ion Channel Gating; Kidney Tubules, Distal; Membrane Transport Proteins; Renin-Angiotensin System; Signal Transduction; Sodium; Sulfate Transporters
PubMed: 26538443
DOI: 10.1152/ajprenal.00400.2015 -
Cureus Jun 2023Primary hyperaldosteronism typically leads to resistant hypertension, hypokalemia, and metabolic alkalosis. Excess aldosterone secretion by the adrenal glands may lead...
Primary hyperaldosteronism typically leads to resistant hypertension, hypokalemia, and metabolic alkalosis. Excess aldosterone secretion by the adrenal glands may lead to heart failure with preserved ejection fraction. Potassium-sparing diuretics and aldosterone antagonists directed to lower excess aldosterone levels may help treat the associated heart failure and lead to control of blood pressure, resulting in improved outcomes. We report a case of a 55-year-old male with poorly controlled hypertension and newly symptomatic heart failure with preserved ejection fraction in the setting of excess aldosterone activity and an adrenal adenoma suggesting primary aldosteronism-induced diastolic heart failure. The biochemical evaluation revealed elevated plasma aldosterone concentrations with low plasma renin activity, diuretic-induced hypokalemia, and metabolic alkalosis. A progressively enlarging left adrenal adenoma was found on abdominal imaging along with resistant hypertension despite the use of multiple antihypertensive medications. Medical management targeted to lower excess aldosterone levels with the use of aldosterone antagonists helped us achieve better blood pressure control and resolution of symptoms of diastolic dysfunction. Treating the underlying pathology helped us improve overt heart failure and may suggest that goal-directed therapy towards the inciting factors may potentially lead to a path to reverse the heart failure symptoms clinically.
PubMed: 37485117
DOI: 10.7759/cureus.40753 -
Frontiers in Pediatrics 2022Bartter syndrome (BS) is a rare tubulopathy that causes polyuria, hypokalemia, hypochloremic metabolic alkalosis, and normotensive hyperreninemic hyperaldosteronism. It... (Review)
Review
Bartter syndrome (BS) is a rare tubulopathy that causes polyuria, hypokalemia, hypochloremic metabolic alkalosis, and normotensive hyperreninemic hyperaldosteronism. It is characterized by locus, clinical, and allelic heterogeneity. Types 1-4 of BS are inherited according to an autosomal recessive pattern, while type 5, which is transient, is X linked. There are specific correlations between the clinical expression and the molecular defect, but since it is a rare disease, such studies are rare. Therapeutic interventions are different, being correlated with types of BS.
PubMed: 35722471
DOI: 10.3389/fped.2022.908655 -
Revista Espanola de Enfermedades... Nov 2021We have read the article by Pérez-Santiago L et al. on the conservative or surgical management of pneumatosis intestinalis (PI). Recently we saw a case of a 18-year-old...
We have read the article by Pérez-Santiago L et al. on the conservative or surgical management of pneumatosis intestinalis (PI). Recently we saw a case of a 18-year-old female diagnosed with anorexia nervosa who presented due to general malaise, asthenia, and inability to walk following an episode of abdominal pain, vomiting and diarrhea (10-15 stools daily, some of them bloody). Physical examination revealed signs of malnutrition and dehydration, and a distended, tender abdomen with no signs of peritoneal irritation. Laboratory chemistry tests revealed macrocytic anemia and metabolic alkalosis. An abdominal CT scan showed pancolonic pneumatosis, with greater involvement of the cecum, ascending and transverse colon, as well as pneumoperitoneum and gas in branches of the superior mesenteric and portal veins.
Topics: Adolescent; Anorexia Nervosa; Female; Humans; Pneumatosis Cystoides Intestinalis; Pneumoperitoneum; Portal Vein; Tomography, X-Ray Computed
PubMed: 34154369
DOI: 10.17235/reed.2021.8013/2021 -
Translational Pediatrics Apr 2019Critical congenital heart disease (cCHD) is the most common reason for acute cardiac failure in the neonatal period. cCHD, defined by systemic low cardiac output (LCO)... (Review)
Review
Critical congenital heart disease (cCHD) is the most common reason for acute cardiac failure in the neonatal period. cCHD, defined by systemic low cardiac output (LCO) and requiring surgery or catheter-based intervention in the first year of life, has an incidence of approximately 15% of CHD and is responsible for up to 25% fatalities of newborn infants. Clinical deterioration develops in most cases due to rapid closure of the ductus arteriosus (DA). Early diagnosis and immediate treatment determinate beneficial outcome. Critical CHD can be classified in duct-dependent systemic flow, duct-dependent pulmonary flow and transposition of the great arteries. The latter two manifest themselves in oxygen resistant cyanosis, whereas CHD with duct-dependent systemic flow may present itself with cardiogenic shock, which can be difficult to differentiate from other causes of shock such as sepsis. Besides prostaglandin therapy for reopening the arterial duct, a balanced parallel pulmonary and systemic circulation should be a therapeutic goal. In CHD with duct-dependent systemic flow a decrease of pulmonary resistance should be avoided; therefore inadequate oxygen therapy, hyperventilation and alkalosis due to excessive treatment of acidosis, should be averted. Volume therapy should be performed carefully. In CHD with duct-dependent pulmonary flow, pulmonary resistance can be decreased, in case of poor pulmonary flow systemic resistance should be increased, mild alkalosis is recommended. Intense volume therapy is in most cases necessary, except if a restrictive atrial communication is present. In addition to intensive care measures, an arsenal of catheter- and surgery-based procedures need to be hold available as back-up for emergency procedures. Transcatheter interventions are nowadays decisive. Atrial-septostomy was the first and still the most utilized high-urgency procedure; DA-stenting is used in prostaglandin-refractory duct stenosis. In the presence of critical aortic valve stenosis, palliation consists of balloon valvuloplasty. In critical aortic coarctation with myocardial failure and no response to prostaglandin, palliative balloon angioplasty may be the method of choice as bridging for corrective surgery.
PubMed: 31161078
DOI: 10.21037/tp.2019.04.06 -
European Journal of Pediatric Surgery :... Dec 2023Normalization of metabolic alkalosis is an important pillar in the treatment of infantile hypertrophic pyloric stenosis (IHPS) because uncorrected metabolic alkalosis...
BACKGROUND
Normalization of metabolic alkalosis is an important pillar in the treatment of infantile hypertrophic pyloric stenosis (IHPS) because uncorrected metabolic alkalosis may lead to perioperative respiratory events. However, the evidence on the incidence of respiratory events is limited. We aimed to study the incidence of peroperative hypoxemia and postoperative respiratory events in infants undergoing pyloromyotomy and the potential role of metabolic alkalosis.
MATERIALS AND METHODS
We retrospectively reviewed all patients undergoing pyloromyotomy between 2007 and 2017. All infants received intravenous fluids preoperatively to correct metabolic abnormalities close to normal. We assessed the incidence of perioperative hypoxemia (defined as oxygen saturation [SpO] < 90% for > 1min) and postoperative respiratory events. Additionally, the incidence of difficult intubations was evaluated. We performed a multivariate logistic regression analysis to evaluate the association between admission or preoperative serum pH values, bicarbonate or chloride, and peri- and postoperative hypoxemia or respiratory events.
RESULTS
Of 406 included infants, 208 (51%) developed 1 or more episodes of hypoxemia during the perioperative period, of whom 130 (32%) experienced it during induction, 43 (11%) intraoperatively, and 112 (28%) during emergence. About 7.5% of the infants had a difficult intubation and 17 required more than 3 attempts by a pediatric anesthesiologist. Three patients developed respiratory insufficiency and 95 postoperative respiratory events were noticed. We did not find a clinically meaningful association between laboratory values reflecting metabolic alkalosis and respiratory events.
CONCLUSIONS
IHPS frequently leads to peri- and postoperative hypoxemia or respiratory events and high incidence of difficult tracheal intubations. Preoperative pH, bicarbonate, and chloride were bad predictors of respiratory events.
Topics: Infant; Humans; Child; Pyloric Stenosis, Hypertrophic; Retrospective Studies; Bicarbonates; Chlorides; Hypoxia; Alkalosis
PubMed: 36417975
DOI: 10.1055/a-1984-9803 -
Indian Journal of Nephrology 2024Respiratory alkalosis during hemodialysis session is a rare complication. We managed two patients with severe respiratory alkalosis, a woman who developed this 75 min...
Respiratory alkalosis during hemodialysis session is a rare complication. We managed two patients with severe respiratory alkalosis, a woman who developed this 75 min after the beginning of the session and a man who developed it about 1 h before the end of the session. In both, the cause was a hypotensive episode, and both hypotension and alkalosis were successfully treated.
PubMed: 38681025
DOI: 10.4103/ijn.ijn_297_22 -
BMJ Case Reports Jan 2021Gitelman syndrome (GS) is an autosomal recessive disease characterised by the presence of hypokalaemic metabolic alkalosis with hypomagnesaemia and hypocalciuria. The...
Gitelman syndrome (GS) is an autosomal recessive disease characterised by the presence of hypokalaemic metabolic alkalosis with hypomagnesaemia and hypocalciuria. The prevalence of this disease is 1-10/40 000. GS is usually associated with mild and non-specific symptoms and many patients are only diagnosed in adulthood. The disease is caused by mutations in the SLC12A3 gene. We present the case of a 49-year-old man referred to a nephrology appointment due to persistent hypokalaemia and hypomagnesaemia. Complementary evaluation revealed hypokalaemia, hypomagnesaemia, metabolic alkalosis, hyperreninaemia, increased chloride and sodium urinary excretion, and reduced urinary calcium excretion. Renal function, remainder serum and urinary ionogram, and renal ultrasound were normal. A diagnosis of GS was established and confirmed with genetic testing which revealed a novel mutation in SLC12A3 (c.1072del, p.(Ala358Profs*12)). This novel mutation extends the spectrum of known SLC12A3 gene mutations and further supports the allelic heterogeneity of GS.
Topics: Genetic Markers; Genetic Testing; Gitelman Syndrome; Humans; Male; Middle Aged; Mutation; Solute Carrier Family 12, Member 3
PubMed: 33462018
DOI: 10.1136/bcr-2020-238097