in 1962 , bartter et al . described a clinical syndrome characterized by hypokalemia , metabolic alkalosis , hyperreninemia , hyperaldosteronism and normal blood pressure .
it occurs mostly in childhood or adolescence , and initial presentation in patients over 40 years of age was very rare .
neverthless , neverthless , the syndrome has aroused great interest in many clinical investigators because it may provide new insights in to renal electrolyte metabolism and the pathophysiology of hypertension .
we recently experienced a case of adult - onset bartter s syndrome who showed hypokalemia , metabolic alkalosis , normotension , hyperreninemia , hyperaldosteronism and juxtaglomerular cell hyperplasia .
a 40-year - old woman was admitted to the chonnam university hospital , with a three months history of severe generalized weakness and fatigue .
the first episode occurred three years ago and spontaneously remitted from it following bed rest , she denied ingestion of licorice , diuretics , laxative or any other medication , and she had no significant nausea , vomiting or diarrhea .
the patient appeared relatively well and had a height of 154 cm and a weight of 52 kg .
the pulse rate was 98/min , the blood pressure 100/70 mmhg , and the respiration rate 18/min .
meq / l for serum potassium , 32.7 meq / l for serum bicarbonate , 86.1 mmhg for pao2 , 48 mmhg for paco2 and 32 mmol / l for total co2 content .
serum sodium averaged 139 meq / l , chloride 85 meq / l , total calcium 5.1 meq / l , ionized calcium 2.0 meq / l , inorganic phosphorus 4.7 mg / dl ,
magnesium 1.6 meq / l , uric acid 13.4 mg / dl and serum osmolality 289 mosl / kg .
the blood urea nitrogen was 44.0 mg / dl , serum creatinine 1.7 mg / dl and creatinine clearance 35 ml / min .
pertinent studies of the blood and serum revealed a normal hemoglobin level , hematocrit value , white blood cell count , total protein , albumin , alkaline phosphatase and transaminase levels .
twenty four - hour urinary excretion of sodium was 183 meq , potassium 67 meq , chloride 247 meq , calcium 120 mg , protein 150 mg , glucose 30 mg and urine amounts 2,300 cc .
the plasma renin activity was 48.2 ng / ml / hr and aldosterone level 34.4 ng / dl .
fena was 2.6% , fecl , 5.7% , fek 55% , and feuric acid 8.2% .
thiazide and furosemide were not detected in her urine by high - performance liquid chromatography at the department of pharmacology , chonnam university medical school , kwangju , korea .
juxtaglomerular hyperplasia of varying degrees and multiple vacuolization of proximal tubule due to chronic hypokalemia were noted ( fig . 1 , 2 ) .
the tubules , interstitium and blood vessels were entirely normal with no scarring or atrophy .
the constellation of hypokalemia , relative hypotension , increased renin activity , increased aldosterone level and juxtaglomerular hyperplasia substantiated the diagnosis of bartter s syndrome . following the renal biopsy ,
the patient had been treated with potassium chloride , 40 meq twice a day , spironolactone , 50 mg three times a day , propranolol , 20 mg three times a day , enalapril , 2.5 mg a day and indomethacin , 25 mg three times a day .
the administration of medications led to an increase in serum potassium to 3.5 to 4.5 meg / l . in association with this improvement in the serum potassium concentration , the patient s muscle strength was rapidly recovered and the patient did well for the following two months ( table 1 ) .
bartter s syndrome consists of hypokalemia due to renal potassium wasting , elevated plasma renin activity and aldosterone secretion , normal blood pressure , hyporesponsiveness of blood pressure to infused angiotensin ii and hyperplasia of granular cells of the juxtaglomerular apparatus of the kidney .
clinically , bartter s syndrome can be divided into at least two groups : one group with an eary ( infancy ) , and the other with a late onset of symptoms .
neonatal bartter s syndrome is characterized by the intrauterine onser of polyuria , leading to polyhydramnios between the 22nd and 24th weeks of gestation . in adults ,
the primary etiolgy of bartter s syndrome is still unknown . with the findings of abnormalities in plasma and urinary prostaglandins , many investigators considered a defect in prostaglandin homeostasis as the primary defect .
the inhibition of chloride transport in the loop of henle by prostaglandins , especially in the face of hyperaldosteronism , would lead to potassium wasting and alkalosis .
however , prostaglandin inhibition does not completely cure the defects in bartter s syndrome . also , continued use of prostaglandin inhibitors may not result in continuous benefit to patients with bartter s syndrome .
stein reviewed the published material and the data derived in his laboratory with bartter s syndrome , and concluded that bartter s syndrome may have more one underlying etiology .
bartter and baehler et al favored primary chloride wasting as the defect of bartter s syndrome .
these hypotheses all consider the resulting potassium deficiency as the cause of the prostaglandin , bradykinin , kallikrein , and vasopressor abnormalities .
sodium , chloride , and potassium losses result in volume depletion , increased aldosterone levels , and metabolic alkalosis .
magnesium depletion causes kaliuresis , diuretics cause potassium and volume depletion and vomiting causes renal potassium wasting and volume depletion .
treatment is generally focused on the repair of hypokalemia by inhibition of the renin - angiotensin - aldosterone or the prostaglandin - kinin system .
potassium supplementation , magnesium repletion , propranolol , spironolactone , prostaglandin inhibitors and conventing enzyme inhibitors all have been advocated , but each has met with limited success . in this case , the patient was diagnosed to be adult - onset bartter s syndrome due to hypokalemia , relative hypotension , increased renin activity , increased aldosterone level and juxtaglomerular hyperplasia . in appropriate medical therapy , a positive potassium balance and an increase in serum potassium concentration . in association with this improvement in the serum potassium concentration ,
the patient s muscle strength was rapidly recovered and the patient did well for the following two months .
 bartter s syndrome is characterized by renal potassium wasting with hypokalemia , metabolic alkalosis , increased renin - angiotensin - aldosterone system , normal blood pressure , resistance to the pressor effects of angiotensin ii and juxtaglomerular cell hyperplasia . 
 most of the cases have been noted in the pediatric age group and adult - onset cases are very rare . 
 we report a case of adult - onset bartter s syndrome . 
INTRODUCTION
CASE REPORT
DISCUSSION