Acquired autoimmune Bartter syndrome in a patient with primary hypothyroidism.

We describe an unusual clinical presentation of autoimmune Bartter syndrome in a patient with primary hypothyroidism. A 65-year-old female patient was admitted with neuromuscular weakness associated with hypokalemia and metabolic alkalosis. She had a suboptimal response to potassium supplementation and potassium-sparing diuretic resulting in re-hospitalization with the same symptoms. A detailed serum and urinary biochemistry analysis in the absence of other causes of potassium wasting helped diagnose Bartter syndrome, a rare entity in adults. An autoimmune profile showed anti-Scl-70 antibody to be positive, although she did not develop other systemic features of the disease. Our patient responded to a steroid-based regimen potassium supplement, Indomethacin, and aldosterone antagonist with remarkable resolution of symptoms and correction of electrolyte derangement. We reviewed the literature to search for similar cases and included twenty-seven full-length publications on acquired and autoimmune causes of Bartter syndrome. Our case highlights the fact that hypokalemia with metabolic alkalosis in an adult patient should prompt clinicians to evaluate for common and uncommon conditions. While assessing for abnormal conditions, acquired Bartter syndrome should be considered if a patient has an underlying autoimmune, endocrine, or connective tissue disease.

Diagnóstico casual de un síndrome de Gitelman / Casual diagnosis of Gitelman's syndrome

El síndrome de Gitelman es una tubulopatía de herencia autosómica recesiva en el que la alteración fundamental se halla en el túbulo distal, concretamente a nivel del cotransportador Na/Cl, sensible a las tiazidas, codificado en el cromosoma 16q. Cursa con alcalosis metabólica con normotensión, hipopotasemia, así como hipomagnesemia e hipocalciuria que la diferencian del síndrome de Bartter. Su diagnóstico puede demorarse hasta la edad adulta ya que los pacientes pueden mantenerse asintomáticos durante largos períodos de tiempo. El tratamiento consiste en suplementos orales de potasio y magnesio, así como también se ha descrito la utilidad de diuréticos ahorradores de potasio e indometacina (AU)

Gitelman's syndrome is a renal tubule disease of recessive autosomal inheritance in which the fundamental alteration is found in the distal tubule, specifically at the level of the Na/Cl cotransporter, is sensitive to thiazides, and coded in chromosome 16q. It is characterised by a metabolic alkalosis with normal blood pressure, hypokalaemia, as well as hypomagnesaemia and hypocalciuria, which separate it from Bartter's syndrome. Its diagnosis can be delayed up to the adult age, as patients may remain asymptomatic for long periods of time. The treatment consists of oral supplements of potassium and magnesium, and the use of potassium-sparing diuretics and indomethacin has also been described (AU)

Hypercalcemia and acute renal failure in milk-alkali syndrome: a case report.

Historically, the milk-alkali syndrome developed as an adverse reaction to the Sippy regimen of milk, cream and alkaline powders as treatment for peptic ulcer disease. The classic description includes hypercalcemia, metabolic alkalosis, and renal failure. Over the past 20 years, milk-alkali syndrome has had a resurgence, as consumption of supplements containing calcium has increased. A 46-year-old man presented to the emergency department after outpatient labs to evaluate his fatigue. He was found to have acute renal failure and hypercalcemia (total serum calcium was 15.9 mg/dL). Subsequent laboratory evaluation excluded both hyperparathyroidism and malignancy as causes. A detailed history led to the diagnosis of milk-alkali syndrome. With hydration and cessation of calcium carbonate ingestion, his renal function and serum calcium levels returned to normal. Physicians should have a high index of suspicion for milk-alkali syndrome in patients with hypercalcemia. Milk-alkali syndrome is no longer a merely a historical curiosity; it is currently the third most common cause of hypercalcemia.

Teneurin carboxy (C)-terminal associated peptide-1 inhibits alkalosis-associated necrotic neuronal death by stimulating superoxide dismutase and catalase activity in immortalized mouse hypothalamic cells.

The teneurins and the teneurin C-terminal-associated peptides (TCAP) are implicated in the regulation of neuron growth and differentiation. However, current observations suggest that TCAP-1 may also have a neuroprotective action during times of pH-induced cellular stress in the brain such as during hypoxia-ischemia and brain alkalosis. To test this hypothesis, we cultured a TCAP-1-responsive mouse hypothalamic cell line, N38, using media buffered at pHs 6.8, 7.4, 8.0 and 8.4 subsequently treated with 100 nM TCAP-1. TCAP-1 significantly inhibited the decline in cell proliferation at pHs 8.0 and 8.4 as determined by direct cell viability assays and decreased the incidence of cells showing necrotic morphology. In addition, TCAP-1 decreased the number of cells undergoing necrosis by 4- to 5-fold as measured by uptake of ethidium homodimer III. Moreover, TCAP-1 significantly decreased the incidence of superoxide radicals and increased superoxide dismutase 1 (SOD1) expression. These results were accompanied by an increase in the SOD copper chaperone expression and increased catalase activity and expression. The results indicate that TCAP may play a neuroprotective role during periods of pH stress by upregulating oxygen radical scavenging systems. Thus, the TCAP-teneurin system may be part of a mechanism to protect neurons during trauma, such as hypoxia and ischemia.

Saline-resistant metabolic alkalosis, severe hypokalemia and hypertension in a 74-year-old woman.

The case of a 74-year-old woman with past history of hypertension and cerebrovascular accident admitted with pneumonia, dehydration, hypernatremia and severe hypokalemic alkalosis is presented. After correction of the hypertonic dehydration, the hypokalemia and alkalosis persisted in spite of aggressive potassium supplementation and the patient became hypertensive. Mineralocorticoid excess was suspected and excluded after extensive endocrinological testing. The use of aldactone failed to revert the abnormalities. Triamterene administration corrected the electrolytes and acid base aberrations, and dramatically improved the blood pressure control. This clinical picture is compatible with the diagnosis of Liddle's syndrome. Our patient exemplifies the unique occurrence of hypokalemic metabolic alkalosis in association with volume contraction at the start of the hospitalization and volume expansion later on her course.

Hypokalaemic alkalosis, acquired Gitelman's and Bartter's syndrome in chronic sialoadenitis.

Two patients with chronic sialoadenitis had features of Bartter's and Gitelman's syndrome, respectively. The main complaints were leg paraesthesiae and acute arthritis. A good response to oral K+ supplementation, allopurinol and low-dose prednisone was obtained. The features of Sjögren's-related renal diseases are reviewed.

[Hypertension caused by licorice consumption]. / Hypertensie door dropgebruik.

In a 38-year-old woman who was hospitalized because of hypertension and hypokalaemic alkalosis, the intake of liquorice (200 g per day) was proven to be the cause. A liquorice provocation test produced all the expected clinical and biochemical abnormalities. Some kinds of liquorice contain glycyrrhetic acid which inhibits the enzyme 11-beta-hydroxysteroid dehydrogenase (e.g. in the kidney) leading to decreased transformation of cortisol into cortisone. The mineralocorticoid action of cortisol causes a drop in serum potassium and an increase in serum sodium concentration, together with a metabolic alkalosis, which in the patient described led to retention of water resulting in weight increase and hypertension.

[Severe metabolic alkalosis with a consciousness disorder]. / Schwere metabolische Alkalose mit Bewusstseinstrübung.

HISTORY AND CLINICAL FINDINGS: A 47-year-old man in a reduced general condition, presumed to be a chronic alcoholic, was hospitalised in a sleepy state and impaired level of consciousness (Glasgow Coma Scale 8). There were no focal neurological deficits, but all proprioceptor reflexes were weak. Body temperature was 36.8 degrees C, blood pressure 90/60 mm Hg, and heart rate 80/min. INVESTIGATIONS: Biochemical tests showed sodium concentration reduced to 121 mmol/l, potassium to 1.83 mmol/l, chloride to 55 mmol/l and, on the next day, phosphate to 0.11 mmol/l. Blood gas analysis demonstrated a noncompensated respiratory alkalosis (pH 7.69, bicarbonate 39.5 mmol/l and a base excess of 20 mmol/l. TREATMENT AND COURSE: The impaired consciousness was thought to be due to the marked alkalosis in combination with hypophosphataemia. The alkalosis was completely removed within 48 hours by administration of Ringer's solution and potassium chloride concentrate, without sodium chloride Phosphate deficit was neutralised with KH2PO4 infusion. Normal consciousness was restored. CONCLUSIONS: Even severe hypochloraemic alkalosis can be quickly reversed with infusion of chloride without sodium Successful treatment with chloride alone excludes alkalosis induced by mineralocorticoids.

Phosphorus magnetic resonance spectroscopic imaging in patients with frontal lobe epilepsy.

Phosphorus magnetic resonance spectroscopic imaging has previously demonstrated localized metabolic abnormalities within the epileptogenic region in patients with temporal lobe epilepsy, including alkalosis, increased inorganic phosphate level, and decreased phosphomonoester levels. We studied 8 patients with frontal lobe epilepsy, finding interictal alkalosis in the epileptogenic region compared to the contralateral frontal lobe in all patients (7.10 +/- 0.05 vs 7.00 +/- 0.06, p < 0.001). Seven patients exhibited decreased phosphomonoester levels in the epileptogenic frontal lobe compared to the contralateral frontal lobe (16.0 +/- 6.0 vs 23.0 +/- 4.0, p < 0.01). In contrast to findings in temporal lobe epilepsy, inorganic phosphate level was not increased in the epileptogenic region. Based on values derived from normal control subjects, 5 patients had elevated pH in the seizure focus and 2 patients had decreased phosphomonoesters while none had abnormalities in the contralateral frontal lobe. These data suggest that magnetic resonance spectroscopy will be useful in the presurgical evaluation of patients with frontal lobe epilepsy.

Chronic metabolic alkalosis in an infant with cystic fibrosis.

A 6-month-old infant suffering from cystic fibrosis is reported. In spite of an apparently appropriate treatment and in absence of respiratory infection, the patient showed progressive anorexia, intermittent vomiting and weight loss. These non-specific signs and symptoms could all be explained by metabolic alkalosis and disappeared immediately after oral supplementation with sodium and potassium chloride. This unusual metabolic complication should be searched for in every cystic fibrosis infant with unexplained anorexia and failure to thrive.

Effect of magnesium depletion and potassium depletion and chlorothiazide on intracellular pH in the rat, studied by 31P NMR.

1. Both dietary magnesium depletion and potassium depletion (confirmed by tissue analysis) were induced in rats which were then compared with rats treated with chlorothiazide (250 mg/kg diet) and rats on a control synthetic diet. 2. Brain and muscle intracellular pH was measured by using a surface coil and [31P]-NMR to measure the chemical shift of inorganic phosphate. pH was also measured in isolated perfused hearts from control and magnesium-deficient rats. Intracellular magnesium status was assessed by measuring the chemical shift of beta-ATP in brain. 3. There was no evidence for magnesium deficiency in the chlorothiazide-treated rats on tissue analysis or on chemical shift of beta-ATP in brain. Both magnesium and potassium deficiency, but not chlorothiazide treatment, were associated with an extracellular alkalosis. 4. Magnesium deficiency led to an intracellular alkalosis in brain, muscle and heart. Chlorothiazide treatment led to an alkalosis in brain. Potassium deficiency was associated with a normal intracellular pH in brain and muscle. 5. Magnesium depletion and chlorothiazide treatment produce intracellular alkalosis by unknown mechanism(s).

Stridor due to drug-induced hypokalaemic alkalosis.

A 76-year-old on long-term Lasix and Pyrogastrone presented with stridor. This became worse with local irritation, e.g. on coughing or during indirect laryngoscopy. Indirect laryngoscopy showed a narrow glottis with an otherwise normal larynx. Blood investigation showed a low serum potassium with a raised bicarbonate level, and a serum calcium level just within the acceptable normal range. A diagnosis of laryngospasm secondary to drug-induced hypokalaemic alkalosis was made. This was treated with the withdrawal of the above drugs and supplementing potassium orally.

Systemic alkalosis and digitalis related arrhythmias.

Reviews of large series of patients with digitalis-induced arrhythmias create a seeming paradox: Hypokalemia is infrequently associated with digitalis-induced arrhythmias but the clinical benefit of supplementation of potassium for most digitalis-induced arrhythmias is obvious. Examination of the electrophysiologic abnormalities induced by digitalis coupled with the electrophysiologic effects dependent on the ratio of intracellular to extracellular concentrations of potassium clarifies the issue. We present evidence that supports additive effects of the toxicity of digitalis and abnormal ratios of concentrations of potassium inside and outside the cardiac cell using metabolic alkalosis as a marker of intracellular potassium depletion. Patients with metabolic alkalosis and normokalemia with "therapeutic" concentrations of digoxin had significantly greater prevalence of arrhythmias than did patients without alkalosis. We presume this effect of alkalosis to be mediated by effects on extra- to intracellular ratios of potassium.

Digoxin toxicity in patients with normokalemic potassium depletion.

Reviews of large series of patients with digitalis-induced arrhythmias create a seeming paradox: Hypokalemia is infrequently associated with digitalis-induced arrhythmias but the clinical benefit of supplementation of potassium for most digitalis-induced arrhythmias is obvious. Examination of the electrophysiologic abnormalities induced by digitalis coupled with the electrophysiologic effects dependent on the ratio intracellular to extracellular concentrations of potassium clarifies the issue. We present evidence that supports additive effects of the toxicity of digitalis and abnormal ratios of concentrations of potassium inside and outside the cardiac cell. We provide guidelines for assessing this crucial ratio of intracellular to extracellular concentrations of potassium to aid the clinician in the diagnosis and effective treatment of digitalis-induced arrhythmias.

Hypokalemic, metabolic alkalosis induced by high-dose ampicillin sodium.

A case of hypokalemic metabolic alkalosis precipitated by high-dose intravenous ampicillin sodium is discussed. Cases of hypokalemic metabolic alkalosis attributable to ampicillin sodium have not been reported previously. There have been reports of this phenomenon associated with high doses of penicillin sodium and carbenicillin disodium. The possible mechanism of antibiotic-induced hypokalemic metabolic alkalosis is discussed. It is suggested that most cases of antibiotic-induced hypokalemia respond to oral or intravenous potassium chloride.

Phosphorus deficiency and hypophosphatemia.

Low serum phosphorus levels, sometimes associated with depletion of phosphorus stores, can engender a variety of serious, often life-threatening physiologic changes. The proximate cause of this dangerous situation is usually medical intervention in such conditions as alcoholism and diabetic ketoacidosis, which can produce a shift of phosphorus within the body unless preventive measures are instituted.

Hypokalemia in children with leukemia in relapse.

Eight children with acute leukemia in relapse were hypokalemic during their hospital course. All had accompanying hypophosphatemia, and three had mild metabolic alkalosis. Potassium chloride supplementation in each case resulted in resolution of the electrolyte and acid-base disturbances. These findings were not present in patients with conditions newly diagnosed or those in remission. The pathogenesis of the electrolyte and acidbase disturbances was not evident and was not related to antibiotic or cytotoxic drug therapy, but may have been related to the patients' poor nutritional status. Seven of the eight patients died within six months of the hypokalemic episode. Hypokalemia may be a common accompaniment of terminal leukemia.

Hypokalemia and metabolic alkalosis resulting from overuse of magnesium oxide.

A 23 year old woman had taken large doses of magnesium oxide (up to 20 to 30g per day) initially for habitual constipation and later for idiopathic edema instead of diuretics, until the concealed abuse was discovered. During the abuse she showed hypokalemia and metabolic alkalosis. When the ingestion was stopped, the electrolyte imbalance recovered within two weeks.