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Aims: To determine the prevalence and the impact on prognosis of metabolic alkalosis (MA) in patients admitted for acute heart failure (AHF)., Methods and Results: The ALCALOTIC is a multicenter, observational cohort study that prospectively included patients admitted for AHF. Patients were classified into four groups according to their acid-base status on admission: acidosis, MA, respiratory alkalosis, and normal pH (reference group for comparison). Primary endpoint was all-cause in-hospital mortality, and secondary endpoints included 30/90-day all-cause mortality, all-cause readmission, and readmission for HF. Associations between endpoints and acid-base alterations were estimated in a multivariate Cox regression model including sex, age, comorbidities, and Barthel index and expressed as hazard ratio (HR) with 95% confidence interval (95% CI). Six hundred sixty-five patients were included (84 years and 57% women), and 40% had acid-base alterations on admission: 188 (28%) acidosis and 78 (12%) alkalosis. The prevalence (95% CI) of MA was 9% (6.8-11.2%). Patients with MA were more women; had fewer comorbidities, better renal function, and higher left ventricle ejection fraction values; and received more treatment with oral acetazolamide during hospitalization and at discharge. MA was not associated with a higher risk of in-hospital mortality and 30/90-day all-cause mortality or readmissions but was associated with a significant increase in readmissions for HF at 30 and 90 days (adjusted HR [95% CI] 3.294 [1.397-7.767], p = 0.006 and 2.314 [1.075-4.978], p = 0.032)., Conclusion: The prevalence of MA in patients admitted for AHF was 9%, and its presence was associated with more readmissions for HF but not with all-cause mortality., (© 2024. Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Hemodialysis solutions typically contain a high alkali concentration designed to counter interdialytic acidosis, but this could result in persistent alkalosis in some patients. The prevalence and significance of persistent alkalosis were therefore examined at four outpatient centers over a 10-year period., Methods: Alkalosis was defined as a pre-dialysis serum [HCO 3 ] ≥ 26 meq/L in >6 months of a 12-month period and was persistent if present in a majority of months thereafter. Control patients had a serum [HCO 3 ] of 19-23 meq/L > 6 of every 12 months. Standard, citrate-containing dialysate was used in all patients without adjustment of bicarbonate concentration., Results: 444 of 1271 patients had alkalosis that persisted in 73. Compared to control patients, persistently alkalotic patients were older, but gender, race, starting weight, comorbidities, and mortality did not differ. Dialysis dose was 7% greater, protein catabolic rate was 11% lower, and interdialytic weight gain was 29% lower, all p < 0.001. Persistently alkalotic patients had double the incidence of cardiac arrhythmias (p = 0.07) and a 20% greater intradialytic blood pressure decrease (p < 0.001)., Conclusions: Alkalosis is common in hemodialysis patients and can be persistent, likely due to decreased protein catabolic rate and increased dialysis dose, and may have detrimental cardiovascular effects., (© 2022 Wiley Periodicals LLC.)

Objective: Uncorrected metabolic alkalosis in infantile hypertrophic pyloric stenosis (IHPS) could lead to perioperative apnea. However, the precise incidence of preoperative respiratory problems and the association with metabolic alkalosis are unknown. Therefore, we aimed to determine the incidence of preoperative respiratory problems in IHPS and to assess the association with metabolic alkalosis., Methods: We retrospectively reviewed all patients diagnosed with IHPS during 2007-2017. Respiratory problems were classified as present or absent. With multivariate logistic regression we analyzed the association between bicarbonate and respiratory problems, corrected for gestational age and birth weight., Results: We included 459 infants, of whom 23 developed preoperative respiratory problems (5.0%). Infants with preoperative respiratory problems were more often female (43.5% vs. 13.3% p = 0.001) and had significantly higher median serum levels of bicarbonate (32.0 mmol/L vs. 30.0 mmol/L), base excess (6.5 mmol/L vs. 5.3 mmol/L) and pCO2 (6.4 kPa vs. 5.9 kPa), compared to infants without respiratory problems. Multivariate analysis of serum bicarbonate and presence of respiratory problems showed an OR of 2.18 per 10 mmol/L (95% CI 1.21-4.71) (p = 0.009). The optimal bicarbonate cutoff point was 25.7 mmol/L (sensitivity 100%, specificity 13.4%)., Conclusion: IHPS with metabolic alkalosis potentially results in preoperative respiratory problems. A lower bicarbonate target before surgery might be recommended and respiratory monitoring should be considered., Level of Evidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Patients with permanent postsurgical hypoparathyroidism, a complication of total thyroidectomy, often require high calcium supplementation with vitamin D to maintain serum calcium levels. The epidemiology of calcium-alkali syndrome (CAS) in patients with hypoparathyroidism after total thyroidectomy remains unclear. This study aimed to investigate the incidence of hypercalcemia, renal impairment, metabolic alkalosis, and CAS in patients treated for presumed hypoparathyroidism after total thyroidectomy., Methods: Twenty-seven patients with neck cancers who underwent total thyroidectomy without parathyroid autotransplantation between January 2010 and October 2013 at our hospital were consecutively included. All patients received calcium lactate and alfacalcidol for postsurgical hypocalcemia. We defined hypercalcemia as a corrected serum calcium level (cCa) ≥10.5 mg/dL, metabolic alkalosis as a difference in serum sodium and serum chloride ([sNa-sCl]) ≥39 mEq/L, and renal impairment as a ≥50% increase in serum creatine and/or ≥35% decrease in estimated glomerular filtration rate (eGFR) compared to baseline., Results: cCa peaked (11.1 ± 1.5 mg/dL) at a median of 326 days (interquartile range 78-869) after surgery. At peak cCa, [sNa-sCl] was significantly higher (p < 0.01), and eGFR was significantly lower (p < 0.01) than that at baseline. Fifteen patients (55.6%) had hypercalcemia, 19 (70.3%) had alkalosis, 12 (44.4%) had renal impairment, and 9 (33.3%) had CAS. Patients with CAS (mean age 67.1 ± 10.8 years) were older than those without CAS (56.7 ± 13.6 years, p = 0.06). The mean dose of alfacalcidol in the CAS group (3.1 ± 1.2 μg/day) was significantly larger than that in the non-CAS group (2.1 ± 1.0 μg/day, p = 0.03)., Conclusions: This retrospective study reveals the high incidence of CAS in patients with hypoparathyroidism after total thyroidectomy. Furthermore, these findings suggest that the serum calcium level, acid-base balance, and renal function should be closely monitored in patients with postsurgical hypoparathyroidism who receive large doses of active vitamin D., (© 2020 S. Karger AG, Basel.)

Introduction: Acid base disorder is a condition characterized by alteration in blood pH by the imbalance between the components of blood leading to a life threatening situation. The main aim of this study was to find the prevalence of acid-base disorders and biochemical findings of such disorders in patients in a tertairy care hospital., Methods: This descriptive cross-sectional study was conducted in Nobel Medical College Teaching Hospital from 1st September, 2018 to 31st August, 2019. Ethical apporoval was taken from Institutional Review Committee. All the patients presented to emergency department, intensive care units and wards were included during the study period. Data were entered and calculations were done in Microsoft Excel, point estimate at 95% Confidence Interval was calculated along with frequency and proportion for binary data., Results: Out of arterial blood gas analysis of 1144 patients, the prevalence of acid base disorders was 718 (62.76%) at 95% Confidence Interval (59.96-65.56%). Simple and mixed acid base disorders were observed in 332 (46.24%) and 386 (53.76%) patients respectively. Respiratory alkalosis was most common among 134 (40.36%) cases in simple acid base disorder whereas metabolic acidosis and respiratory alkalosis was most common among 204 (52.85%) in mixed acid base disorder. All types of disorders were observed more in elderly people (41-60 and >60 age group) than other age groups., Conclusions: Acid base disorder was found to be more common in very ill patients in emergency and intensive care units. Mixed acid base disorder was the most common with male and elderly patients in predominance.

Background: The etiology of sudden cardiac death in patients with end-stage renal disease (ESRD) on hemodialysis (HD) is largely unknown, though there is evidence to suggest that metabolic alkalosis induced by HD with a high-bicarbonate dialysate/prescription may play a role., Methods: We investigated the effects of metabolic alkalosis induced by HD with an acetate-containing bicarbonate-buffered dialysate on frequency of ventricular arrhythmia in 47 patients with ESRD on chronic HD using 48-h Holter monitoring in 3 phases: intra-HD, post-HD day 1, and post-HD day 2. Serum levels of bicarbonate, calcium, and potassium along with hemodynamics were measured pre-HD, post-HD, 20-h post-HD, and 44-h post-HD. Correlations were performed to verify the association between bicarbonate prescription and change in serum bicarbonate levels post-HD and to determine if the HD-induced change in serum bicarbonate level (metabolic alkalosis) had any direct association with ambient ventricular arrhythmia (premature ventricular contractions per hour) or indirect associations with ambient ventricular arrhythmia by affecting electrolytes or hemodynamics that are known to increase the risk of ventricular arrhythmia., Results: Mean pre-HD serum bicarbonate level was 21.3 mEq/L. Dialysate bicarbonate prescription (mean of 36.4 mEq/L) correlated with changes in serum bicarbonate levels immediately post-HD 26.7 mEq/L (r = 0.46, p < 0.01), 20-h post-HD 25.2 mEq/L (r = 0.38), and 44-h post-HD 23.2 mEq/L (r = 0.35, p = 0.01). No statistically significant correlations were found between the post-HD change in serum bicarbonate levels (metabolic alkalosis) with ambient ventricular arrhythmia, changes in serum calcium, potassium, or hemodynamics in any phase., Conclusions: High-bicarbonate dialysate prescription is associated with metabolic alkalosis following the HD procedure. A mild metabolic alkalosis induced by HD with an acetate-containing bicarbonate-buffered dialysate solution had no direct association with ambient ventricular arrhythmia on Holter monitoring and was not associated with changes in hemodynamics or changes in serum total calcium or potassium levels. This study helps to provide guidance for the safe use of high bicarbonate dialysate/prescription in patients with ESRD on HD., (© 2019 S. Karger AG, Basel.)

Objective: To evaluate the incidence of hypochloremic metabolic alkalosis (HCMA) in dogs and cats in the ICU that had intermittent nasogastric tube (NGT) aspiration for up to 36 hours., Design: Prospective cohort study (December 2013 to October 2014)., Setting: Privately owned emergency and referral teaching hospital., Animals: Forty-nine client-owned dogs and 16 client-owned cats., Interventions: Patients wherein NGT placement was recommended and client consent was obtained were included in the interventional group. Those with an NGT placed (NGT group) had the NGT aspirated every 4 hours. Patients for whom placement of a NGT was declined by the owner served as a reference group (NoNGT). Venous blood gas and electrolyte values were obtained every 12 hours., Measurements and Main Results: Thirty-five dogs and cats had an NGT placed. Thirty dogs and cats did not have an NGT placed. The serum venous blood gas and electrolyte changes were compared over time within the NGT group and between the NGT and NoNGT groups. No cases developed HCMA. In the NGT group, blood pH increased over time. There was no significant difference between the NGT and the NoNGT group in the average value of pH, HCO 3 - , base excess, chloride, or corrected chloride. Serum venous blood gas, chloride, and corrected chloride changes were not associated with the volumes of gastric fluid aspirated over time., Conclusions: In this small population of dogs and cats, intermittent NGT aspiration was not associated with the development of HCMA over a period of up to 36 hours after NGT placement., (© Veterinary Emergency and Critical Care Society 2018.)

Pseudo-Bartter Syndrome (PBS), although quite common in patients with cystic fibrosis (CF), is often missed as simple dehydration or Bartter syndrome. This study was performed in patients with PBS to compare the pattern and course of the disease with those with CF not manifesting with this syndrome. All patients with CF who attended the respiratory clinic at Queen Rania Al-Abdallah Hospital from January 2000 to April 2010 were included in this retrospective case-control study. A specially formulated data sheet was used and those with PBS and those not having the syndrome were identified. A total of 110 patients (51% female) with CF with a median age of seven years were followed-up. Eighteen (16.3%) of them had one or more episodes of PBS. The median follow-up period was 6.2 years. All the episodes occurred during summer and in infancy. Median age of the initial episode of PBS was three months. One-third of them were initially followed at the nephrology clinic. Three patterns of PBS were identified: single episode in three (16.6%) patients, recurrent in 12 (66.6%) patients and chronic in three (16.6%) patients. Early colonization of Pseudomonas spp before 1 st birthday was seen in 44% patients with PBS compared with 12% in other CF patients (P-value = 0.0075). The total number of colonized patients and other CF features at the time of the study did not differ significantly among patients, although the mean Shwachman-Kulczycki score is significantly lower in those with recurrent PBS (69 compared with 85 in other CF patients). Gene mutation was identified in only 30% of the entire cohort. PBS is common in patients with CF, and it should be kept in mind in any patient with hypotonic dehydration and metabolic alkalosis. Recurrent pattern is associated with earlier Pseudomonas colonization.

Underlying causes of metabolic alkalosis may be evident from history, evaluation of effective circulatory volume, and measurement of urine chloride concentration. However, identification of causes may be difficult for certain conditions associated with clandestine behaviors, such as surreptitious vomiting, use of drugs or herbal supplements with mineralocorticoid activity, abuse of laxatives or diuretics, and long-term use of alkalis. In these circumstances, clinicians often are bewildered by unexplained metabolic alkalosis from an incomplete history or persistent deception by the patient, leading to misdiagnosis and poor outcome. We present a case of severe metabolic alkalosis and hypokalemia with a borderline urine chloride concentration in an alcoholic patient treated with a thiazide. The cause of the patient's metabolic alkalosis eventually was linked to surreptitious ingestion of baking soda. This case highlights the necessity of a high index of suspicion for the diverse clandestine behaviors that can cause metabolic alkalosis and the usefulness of urine pH and anion gap in its differential diagnosis., (Copyright © 2012 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Posthypercapnic alkalosis (PHA) is frequently overlooked as a complication of mechanical ventilation in patients with exacerbation of chronic obstructive pulmonary disease (COPD). The current study was conducted to determine the incidence, risk factors for development and effect on outcome of PHA. Eighty-four patients (62 +/- 11 years, range 42-78 years, M:F 58: 26) with exacerbation of COPD with underlying chronic hypercapnic respiratory failure requiring mechanical ventilation were included in a retrospective fashion. PHA was defined as static or rising serum bicarbonate levels, 72 hours or more after return of PaCO2 to baseline, with concurrent pH > 7.44. Development of PHA was noted in 17 patients (20.2%). Corticosteroid use >or=10 days during the hospital stay was an independent risk factor for development of PHA (Adjusted OR, 95% CI: 9.4, 1.6-55.3; P = 0.013). Development of PHA was associated with an increased incidence of ventilator dependence (64.7% vs. 37.3%, OR, 95% CI: 3.1, 1.1-9.4, P = 0.04) and duration of ICU stay (14.7 +/- 6.7 vs. 9.5 +/- 5.9, P = 0.01) but no increase in hospital mortality (43.3% vs. 41.2%, P = NS). It is concluded that PHA is a common complication in patients with exacerbation of COPD requiring mechanical ventilation and is associated with increased incidence of ventilator dependence and ICU stay.

Background: Intravenous sodium bicarbonate (SB) administration during cardiopulmonary arrest (CPA) is intended to counteract lactic acidosis due to hypoxia, poor perfusion, and anaerobic metabolism. Despite a lack of documented efficacy and a level III recommendation from the American Heart Association, SB is widely used during resuscitation events. SB has both theoretical and measurable adverse effects. Excess or poorly timed administration during a CPA may elevate a patient's pH, inducing alkalemia. Despite decades of controversy surrounding use of this drug, the prevalence of SB-induced alkalemia has not been previously documented., Objective: To estimate the prevalence of SB-induced alkalemia in inpatients after CPA and to investigate the pattern of SB administration., Methods: Medical records were retrospectively reviewed with attention to SB administration and arterial blood gas (ABG) data. After application of inclusion and exclusion criteria to 264 CPA patients, the study group comprised 88 patients. When measured, if PCO(2) and pH were above normal limits after SB administration, we concluded that SB contributed to the alkalemia., Results: Twenty-seven (31%) patients received SB without any ABG data, and 70 (79%) patients received at least one empiric SB dose. Of the 61 patients with ABG data, alkalemia occurred in 10, a prevalence of 16%. Administration of SB increased pH in only 9 (15%) other CPA patients and had no effect in the 42 (69%) remaining patients., Conclusions: Administration of SB during CPA was causally linked with inducing alkalemia in 16% of patients. Early collection of ABG samples may assist in optimizing pH during CPA and thus reduce unwarranted empiric use of SB.

Regional citrate anti-coagulation for continuous renal replacement therapy chelates calcium to produce the anti- coagulation effect. We hypothesise that a calcium-free replacement solution will require less citrate and produce fewer metabolic side effects. Fifty patients, in a Medical Intensive Care Unit of a tertiary teaching hospital (25 in each group), received continuous venovenous hemofiltration using either calcium-containing or calcium-free replacement solutions. Both groups had no significant differences in filter life, metabolic alkalosis, hypernatremia, hypocalcemia, and hypercalcemia. However, patients using calcium-containing solution developed metabolic alkalosis earlier, compared to patients using calcium-free solution (mean 24.6 hours,CI 0.8-48.4 vs. 37.2 hours, CI 9.4-65, P = 0.020). When calcium-containing replacement solution was used, more citrate was required (mean 280 ml/h, CI 227.2-332.8 vs. 265 ml/h, CI 203.4-326.6, P = 0.069), but less calcium was infused (mean 21.2 ml/h, CI 1.2-21.2 vs 51.6 ml/h, CI 26.8-76.4, P < or = 0.0001).

Renal failure is common among the long-term care (LTC) elderly. Little is known about the acid/base status of these patients. The aim of this study is to evaluate the relationship between the acid base status and renal function in a representative group of skilled nursing patients and relate it to their feeding status. LTC elderly patients, in stable clinical condition, 50 on naso-gastric tube (NGT) feeding, 40 orally fed (OF), were recruited to this study. As controls, we studied a group of 30 elderly independent, ambulatory patients admitted to the acute geriatric departments of the hospital for different causes which were not related to their acid-base status. Venous blood was taken for the routine tests and blood gases. In the LTC study groups a 24-h urine collection was examined for biochemical parameters and calculations of all clearances. Glomerular filtration rate (GFR) was estimated by the Cockroft and Goult and MDRD formulas. Renal function was similar in the two main study groups. Daily secretion of sodium and chloride were 50% lower in the NGT fed patients (p<0.001). The LTC elderly patients had significantly higher venous pH values, with no differences in pCO(2) or HCO(3). An alkalotic state (pH>7.45) was found in 13.6% of them (18% in the NGT and 6.5% in the OF) while none of the independent elderly had such values (p<0.05). Similarly, HCO(3)>34 was found in 12% of the LTC elderly versus none in the independents (p=0.06). Values of pO(2) and O(2) saturation were significantly higher in the nursing elderly and mainly those fed by NGT. Hemoglobin levels had a significantly negative correlation with the pH (r=-0.3, p<0.002). In conclusion, unexpected metabolic alkalosis was found in a group of skilled nursing patients, more prominent in those fed by NGT. This finding warrants the inclusion of routine pH determination in patients whenever pharmacokinetic considerations are essential.

In 2003, occasional military patients with hyponatraemia, hypokalaemia and alkalosis were encountered in Iraq. Development of central pontine myelinolysis in one patient indicated treatment should be cautious. Two years later, heat illness continued to occur during the very hot summer months and 23 cases were admitted to a British military field hospital near Basra, Iraq. Incidence was < 0.15% of deployed personnel per summer month. Serum sodium and potassium concentrations were directly (r = 0.66, p = 0.0002) and serum sodium and bicarbonate concentrations inversely (r= -0.64, p = 0.002) correlated. The magnitude of these changes was unrelated to the glomerular filtered load of sodium. While blood pressure was undiminished, estimated glomerular filtration rate was reduced. These electrolyte changes were compatible with secondary hyperaldosteronism but field conditions constrained further investigation. Hyponatraemia was probably due to salt deficiency rather than overhydration. In some military personnel summer salt supplementation could be essential during operations in hot countries.

Objective: To characterize serum biochemical abnormalities in goats with uroliths., Design: Retrospective case-control series., Animals: 107 male goats with uroliths and 94 male goats with various nonrenal diseases (controls)., Procedures: For male goats, results of serum biochemical analyses collected from 1992 through 2003 were retrieved from computerized records, as were signalment, clinical diagnoses, and discharge status. Results of analyses for BUN, creatinine, phosphorus, calcium, Na, K, Cl, total CO2, anion gap, and glucose were compared between goats with uroliths and control goats., Results: Goats with uroliths had higher mean BUN, creatinine, total CO2, K, and glucose concentrations and lower mean phosphorus, Na, and Cl concentrations than control goats, with no difference in mean calcium concentration and anion gap. Goats with uroliths had higher frequency of azotemia, hypophosphatemia, hypochloridemia, and increased total CO2 and lower frequency of decreased total CO2 than control goats. Urolithiasis occurred more frequently in castrated males than in sexually intact males and in dwarf African breeds than in other breeds., Conclusions and Clinical Relevance: Goats with uroliths often had hypophosphatemia at admission. Hypochloridemic metabolic alkalosis was the most common acid-base disorder. Rupture in the urinary tract system was associated with increased prevalence of hyponatremia and hyperkalemia. Clinicians should be aware of these abnormalities when determining fluid therapy.

A 48-year-old man with diabetes mellitus and hypertension was found to have a thoracic right-sided ectopic kidney with a sensorineuronal hearing loss and tubular abnormalities such as hyponatremia, hypokalemia, metabolic alkalosis with microalbuminuria. He was treated with ACEI and sodium and potassium supplements. However, this only partially corrected his metabolic abnormalities.

Introduction: Metabolic alkalosis is a commonly encountered acid-base derangement in the intensive care unit. Treatment with the carbonic anhydrase inhibitor acetazolamide is indicated in selected cases. According to the quantitative approach described by Stewart, correction of serum pH due to carbonic anhydrase inhibition in the proximal tubule cannot be explained by excretion of bicarbonate. Using the Stewart approach, we studied the mechanism of action of acetazolamide in critically ill patients with a metabolic alkalosis., Methods: Fifteen consecutive intensive care unit patients with metabolic alkalosis (pH > or = 7.48 and HCO3- > or = 28 mmol/l) were treated with a single administration of 500 mg acetazolamide intravenously. Serum levels of strong ions, creatinine, lactate, weak acids, pH and partial carbon dioxide tension were measured at 0, 12, 24, 48 and 72 hours. The main strong ions in urine and pH were measured at 0, 3, 6, 12, 24, 48 and 72 hours. Strong ion difference (SID), strong ion gap, sodium-chloride effect, and the urinary SID were calculated. Data (mean +/- standard error were analyzed by comparing baseline variables and time dependent changes by one way analysis of variance for repeated measures., Results: After a single administration of acetazolamide, correction of serum pH (from 7.49 +/- 0.01 to 7.46 +/- 0.01; P = 0.001) was maximal at 24 hours and sustained during the period of observation. The parallel decrease in partial carbon dioxide tension was not significant (from 5.7 +/- 0.2 to 5.3 +/- 0.2 kPa; P = 0.08) and there was no significant change in total concentration of weak acids. Serum SID decreased significantly (from 41.5 +/- 1.3 to 38.0 +/- 1.0 mEq/l; P = 0.03) due to an increase in serum chloride (from 105 +/- 1.2 to 110 +/- 1.2 mmol/l; P < 0.0001). The decrease in serum SID was explained by a significant increase in the urinary excretion of sodium without chloride during the first 24 hours (increase in urinary SID: from 48.4 +/- 15.1 to 85.3 +/- 7.7; P = 0.02)., Conclusion: A single dose of acetazolamide effectively corrects metabolic alkalosis in critically ill patients by decreasing the serum SID. This effect is completely explained by the increased renal excretion ratio of sodium to chloride, resulting in an increase in serum chloride.

A 56-year-old mentally retarded Japanese woman (intelligence quotient: 49) was admitted to our hospital with the chief complaints of headache, dizziness, vomiting, and lower limb paralysis. Laboratory tests showed severe hypokalemia, metabolic alkalosis, hypomagnesemia, and hypocalciuria. These findings suggested a diagnosis of Gitelman's syndrome (GS). We examined the thiazide-sensitive Na-Cl cotransporter (TSC) gene for the mutations that can be responsible for Gitelman's syndrome, and confirmed the diagnosis. After potassium and magnesium supplementation, her paralysis improved dramatically. The marriage of her parents was consanguineous. She had nine siblings (all with mental retardation), among whom five had died of unknown causes during childhood. Familial mental retardation has never been detected before in Gitelman's syndrome. Here we report a rare case of Gitelman's syndrome with familial mental retardation.

Background: and study objectives: Patients with end-stage cystic fibrosis (CF) develop respiratory failure and hypercapnia. In contrast to COPD patients, altered electrolyte transport and malnutrition in CF patients may predispose them to metabolic alkalosis and, therefore, may contribute to hypercapnia. The aim of this study was to determine the prevalence of metabolic alkalosis in adults with hypercapnic respiratory failure in the setting of acute exacerbations of CF compared with COPD., Design: Levels of arterial blood gases, plasma electrolytes, and serum albumin from 14 consecutive hypercapnic CF patients who had been admitted to the hospital with a respiratory exacerbation were compared with 49 consecutive hypercapnic patients with exacerbations of COPD. Hypercapnia was defined as a PaCO(2) of > or = 45 mm Hg., Results: Despite similar PaCO(2) values, patients in the CF group were significantly more alkalotic than were those in the COPD group (mean [+/- SD] pH, 7.43 +/- 0.03 vs 7.37 +/- 0.05, respectively; p < 0.01). A mixed respiratory acidosis and metabolic alkalosis was evident in 71% of CF patients and 22% of COPD patients (p < 0.01). The mean concentrations of plasma chloride (95.1 +/- 4.9 vs 99.8 +/- 5.2 mmol/L, respectively; p < 0.01) and sodium (136.5 +/- 2.8 vs 140.4 +/- 4.5 mmol/L, respectively; p < 0.01) were significantly lower in the CF group, and the levels of serum albumin were significantly reduced (27.4 +/- 5.8 vs 33.7 +/- 4.8 mmol/L, respectively; p < 0.01)., Conclusion: Metabolic alkalosis contributes to hypercapnic respiratory failure in adults with acute exacerbations of CF. This acid-base disturbance occurs in conjunction with reduced total body salt levels and hypoalbuminemia.

Background: Metabolic alkalosis (MA) is common after orthotopic liver transplantation (OLT)., Methods: The study was conducted to identify factors associated with MA after 285 OLTs. MA, defined as total carbon dioxide content of 30 mEq/L or greater, developed in 115 patients (40%) within the first 3 postoperative days., Results: By univariate analysis, patients with MA had a greater preoperative carbon dioxide content (24.4 +/- 3 versus 22.9 +/- 2.9 mEq/L; P < 0.0001) and hematocrit (35% +/- 5% versus 33% +/- 6%; P < 0.02), but lower creatinine (0.9 +/- 0.5 versus 1.2 +/- 1.2 mg/dL; P < 0.001) and blood urea nitrogen levels (15 +/- 12 versus 19 +/- 17 mg/dL; P < 0.001) compared with controls. Patients with MA were administered more citrate intraoperatively compared with controls (6.2 +/- 5.2 versus 4.5 +/- 3.6 mEq/kg of body weight; P < 0.02). Patients with MA had a lower postoperative potassium level (3.7 +/- 0.4 versus 4 +/- 0.5 mEq/L; P < 0.0001) and cumulative fluid balance (-0.66 +/- 1.87 versus +0.003 +/- 3.9 L; P < 0.007) compared with controls. By multivariate analysis, preoperative carbon dioxide content (odds ratio, 1.19; 95% confidence interval [CI], 1.08 to 1.31 per mEq/L), creatinine level (odds ratio, 0.61; 95% CI, 0.39 to 0.96 per mg/dL), intraoperative administered citrate (odds ratio, 3.35; 95% CI, 1.71 to 6.53 per 10 mEq/kg body weight), and postoperative potassium level (odds ratio, 0.32; 95% CI, 0.18 to 0.57 per mEq/L) were independently associated with MA. MA was not associated with increased hospital mortality (7.8% versus 8.2%, MA versus controls). However, patients with MA spent more time on mechanical ventilation than controls (5 +/- 0.8 versus 3 +/- 0.6 days; P < or = 0.03)., Conclusion: Preoperative total carbon dioxide content, renal function, intraoperative administered citrate, and postoperative potassium level are independently associated with MA after primary OLT., (Copyright 2002 by the National Kidney Foundation, Inc.)

Ectopic adrenocorticotropic hormone (ACTH) and/or corticotropin-releasing hormone (CRH) are associated with a growing list of tumors. We report a 69-year-old white man with a history of high-grade prostate carcinoma and widely metastatic adenocarcinoma who presented with metabolic alkalosis, hypokalemia, and hypertension secondary to ectopic ACTH and CRH secretion. Laboratory values were consistent with hypokalemia and metabolic alkalosis. Markedly elevated serum cortisol (135 microg/dL), ACTH (1,387 pg/dL), CRH (69 pg/dL), and urine free cortisol (16,276 microg/24 h) levels were found. Chest computed tomographic (CT) scan showed small noncalcified parenchymal densities; however, bronchoscopy and bronchoalveolar lavage washings were unremarkable for a neoplastic process. Abdominal CT scan and magnetic resonance imaging showed multiple small liver lesions and multiple thoracic and lumbar intensities consistent with diffuse metastatic disease. Histological analysis of a biopsy specimen from the thoracic spine showed an undifferentiated adenocarcinoma consistent with a prostate primary tumor. The severe metabolic alkalosis secondary to glucocorticoid-induced excessive mineralocorticoid activity was treated with potassium supplements, spironolactone, and ketoconazole. In this case report, we describe an unusual tumor associated with ectopic ACTH and CRH production and the pharmacodynamic relationship of plasma cortisol levels and urinary cortisol excretion with ketoconazole treatment.

The acid-base balance of 199 patients with cystic fibrosis, seen from 1987 through 1992 at the Bern Outpatient Clinic, were evaluated. Simple metabolic alkalosis was demonstrated in 16 and mixed metabolic alkalosis and respiratory acidosis in 9 patients. When compared with 10 patients with simple respiratory acidosis and 16 with normal hydrogen ion balance, those with simple metabolic alkalosis were significantly younger. The need for pancreatic enzymes was significantly higher and the relative underweight significantly more severe in patients with either simple or mixed metabolic alkalosis and respiratory acidosis. The results indicate the rather common occurrence of chronic metabolic alkalosis in cystic fibrosis. It is observed in young patients, in patients who need high doses of pancreatic enzymes and in the those with poor nutritional status.

Congenital chloride diarrhea (CCD) is a common metabolic disorder in Saudi children with an incidence of 1 in 5,500. The present retrospective study from Saudi Arabia, over 7.5 years, presents the clinical, biochemical, and treatment details in 10 children with CCD. The perinatal characteristics of maternal polyhydramnios, prematurity, abdominal distention, and diarrhea were seen in 100% and hyperbilirubinemia in 90% of patients. Hypokalemic hypochloremic metabolic alkalosis was a feature in 50% of the children and acidosis in both neonates. Fecal chloride greater than 100 mmol/L in 100% and fecal chloride greater than the sum of fecal sodium and potassium were found in 55% of patients. The mean age at the time of diagnosis was 10 months and the mean duration of follow-up for the group was 38 months (range 2-89 months). Eight of the nine patients treated with continuous oral electrolyte solution demonstrated "catch-up" physical growth. Mental subnormality, growth retardation, and renal impairment were seen in one patient who failed to comply with the treatment. Cerebral palsy in another child was due to neonatal intraventricular hemorrhage.

Objective: To define the frequency of metabolic alkalosis and its pathogenesis in children after open-heart surgery., Design: Retrospective chart review., Setting: Multidisciplinary, tertiary, pediatric intensive care unit., Patients: Fifty-six consecutive children undergoing open-heart surgery., Measurements and Main Results: Metabolic alkalosis occurred in 29 (52%) of 56 patients. Seventy-two percent of patients < 12 months of age developed metabolic alkalosis as compared with 30% of patients > 12 months of age (p < .01 by chi-square). Patients developing metabolic alkalosis were younger, received more furosemide, had lower serum chloride concentrations, and underwent longer cardiopulmonary bypass times than nonmetabolic alkalosis patients. By stepwise multiple linear regression analysis, only age (p < .05) and serum chloride concentrations (p < .001) had independent correlations with the development of metabolic alkalosis; both variables had inverse correlations with arterial pH (r2 = .42). Patients with metabolic alkalosis also developed significantly (p < .01 by two tailed Student's t-test) lower serum ionized calcium concentrations (4.2 +/- 0.5 mg/dL [1.05 mmol/L]) as compared with nonmetabolic alkalosis patients (4.6 +/- 0.4 mg/dL [1.15 mmol/L])., Conclusions: Postoperative metabolic alkalosis occurs frequently in children undergoing open-heart surgery. Chloride depletion seems to be the predominant factor in the pathogenesis of metabolic alkalosis. Younger age can serve as a positive predictor for the development of metabolic alkalosis in this subset of patients.

The difference in death rate among medical and surgical inpatients having alkalemia is incompletely defined. This prospective study was done to identify the incidence, mortality, and clinical factors that resulted in a poor outcome in both groups of patients. We screened a total of 10,811 consecutive arterial blood gas values; in 4,427 (40.9%) the pH was greater than 7.44, and in 2,577 (23.8%) the pH was greater than 7.48. The study group consisted of 409 patients (213 medical, 196 surgical) whose pH value was greater than 7.48; 253 of these patients (61.9%) had pure respiratory alkalosis, 120 (29.3%) had mixed respiratory and metabolic alkalosis, 27 (6.6%) had a mixed acid base disorder, and nine (2.2%) had pure metabolic alkalosis. Overall group mortality was 27.9% and increased as pH values rose, reaching 48.5% when the pH was greater than 7.60. The overall mortality among medical patients (36.6%) exceeded that among the surgical patients (12.4%) (P less than .05), an observation that applied over a wide range of pH values. Patients having mixed respiratory and metabolic alkalosis had a significantly poorer outcome (44.2% mortality) than the alkalemic group as a whole (27.9% mortality) (P less than .05). Alkalemia-associated illnesses are common in hospitalized patients and are associated with high mortality in both medical and surgical patients, though the death rate is higher among medical patients. Mixed respiratory and metabolic alkalosis appears to be associated with a particularly poor prognosis.

Analysis of 13,430 arterial blood samples demonstrated that metabolic alkalemia was the most common acid-base disturbance encountered in the hospital setting, being present in 51% of patients with abnormal acid-base status. Respiratory alkalemia was encountered in 29%, respiratory acidemia in 27%, and metabolic acidemia in only 12%. Evaluation of those blood gases with metabolic alkalemia, using 95% confidence-limit bands for acid-base disturbances, showed that the metabolic alkalemia was pure in 70% of the cases, mixed with respiratory acidemia in 18%, and combined with respiratory alkalemia in 12%. The adverse effects of alkalemia may be subtle but often significant. Alveolar hypoventilation in response to metabolic alkalemia, without evidence of primary respiratory disease, was more frequent than is generally suspected. This hypoventilation often directly results in hypoxemia, as well as atelectasis with worsened ventilation/perfusion match-up. Alkalemia shifts the oxyhemoglobin dissociation curve to the left, increasing hemoglobin's affinity for oxygen, thus, limiting oxygen release at the tissue level. The presence of metabolic alkalemia makes it more difficult to wean patients from assisted ventilation. A clearer understanding of the frequency and significance of metabolic alkalemia should lead to more appropriate therapy to prevent or correct this acid-base disturbance and should result in a decreased morbidity and mortality in critically ill patients.

A prospective clinical study was performed on 293 patients, in order to elucidate the abnormalities in acid-base balance following general surgery. Six arterial blood gas and pH determinations were taken from each patient before surgery and on postoperative days zero, one, three, five and seven. A total of 1699 determinations were obtained. Although the majority of patients (87.5 per cent) had a normal acid-base balance before surgery, a postoperative metabolic alkalosis was seen in 50.5 per cent of the patients. However, there was an extremely low incidence of other postoperative acid-base abnormalities, apart from a transient increase in metabolic acidosis on the operative day. A significantly high mortality rate (32.3 per cent) was observed in 31 patients who had continuous metabolic alkalosis during the postoperative period. An excessive bicarbonate load resulting from the administration of fresh frozen plasma following surgery was strongly suggested as one of the major causes of postoperative metabolic alkalosis. Further investigation is required to elucidate the mechanism of the generation of metabolic alkalosis induced by the postoperative bicarbonate load in surgical patients.