A case of isolated hypoaldosteronism 5 month old male infant was presented. He was admitted to the pediatric ward with the chief complants of frequent vomiting, dehydration, lethargy and failure to thrive. The diagnosis was established by salt-losing manifestation, laboratory fiding and good response after salt-retaining steroid therapy. A brief review of related literatures were also presented.
Dehydration ; Diagnosis ; Failure to Thrive ; Humans ; Hypoaldosteronism ; Infant ; Lethargy ; Male ; Vomiting

Child ; Female ; Humans ; Hypoaldosteronism ; complications ; Kidney Calculi ; complications

BACKGROUND: TMP/SMX has been shown to cause hyperkalemia in a few outpatients on standard-dose. This prospective study was aimed at investigating other associated factors inducing clinically important hyperkalemia in outpatients on standard-dose of TMP/SMX. METHODS: Age-matched diabetic(n=22) and non-diabetic (n=20) patients with UTI on standard dose of TMP/SMX for 5 days were given acute oral intake of 40 mEq of potassium chloride(KCl). RESULTS: Before the intake of TMP/SMX, basal levels of serum potassium(K), serum BUN and creatinine, plasma renin activity(PRA), aldosterone(PA), and transtubular potassium gradient(TTKG) were comparable between diabetic and non-diabetic subjects. Also after TMP/SMX was taken, all parameters didnt reveal any overt changes except a slightly increased serum K but not significantly (from 4.20+/-0.15 to 4.14+/-0.21mEq/L in non-diabetics; from 4.13+/-0.18 to 4.25+/-0.13mEq/L in diabetics). Following acute oral KCl load, however, the peak increases of serum K changes were significantly higher in diabetics compared to non-diabetics(0.34 0.06 vs 0.62 0.09mEq/L, p<0.01). Furthermore, 8 out of 22 diabetics but none of non-diabetics after acute KCl load developed hyperkalemia(> 5.0 mEq/L). After KCl load, PRA did not show any significant changes, whereas PA was increased simultaneously with the increments of serum K in both diabetic subgroups hyperkalemic(n=8) and normokalemic (n=14) diabetics. But increment was blunted in hyperkalemic diabetic subgroup. TTKG was increased prominently in normokalemic diabetic subgroup(9.20 from 4.50), while it was slightly increased in hyperkalemic diabetic subgroup(4.63 from 3.79mEq/L). There was statistical difference between two subgroups(p < 0.05). In conclusion, Besides the known effect of blocking sodium channels in distal K secreting cells by TMP/SMX, insulinopenia(DM). Hypoaldosteronism with its decreased tubular bioactivity, and increased exogenous K intake in concert could cause clinically overt hyperkalemia on standard-dose of TMP/SMX. When standard- dose of TMP/SMX is administered to patients with deranged K homeostasis, especially to diabetics with hypoaldosteronism, blood K level should be monitored meticulously to avoid hyperkalemia.
Creatinine ; Diabetes Mellitus ; Homeostasis ; Humans ; Hyperkalemia ; Hypoaldosteronism ; Outpatients* ; Plasma ; Potassium* ; Prospective Studies ; Renin ; Sodium Channels

Type IV renal tubular acidosis (RTA) is due to renal tubular bicarbonate wasting associated with mineralocorticoid deficiency. In its five subtypes, IV-4 is due to pseudohypoaldosteronism (PHA) evidenced by increased plasma renin and aldosterone. PHA is believed to result from distal tubular unresponsiveness to circulating aldosterone and has normal renal and adrenal fuction. Hypoaldosteronism can easily be suspected when the patient shows typical electrolyte imbalance (hyponatremia coupled with hyperkalemia) and the diagnosis of PHA is confirmed by elevated serum aldosterone level. But some patients of PHA show negligible electrolyte imbalance, thus metabolic acidosis is a sole abnormal finding in routine laboratory examination. We experienced a case of IV-4 RTA in a 2-month-old male infant who presented with normal anion gap-metabolic acidosis as a sole abnormal finding in routine laboratory examination. RTA was suspected and the test of urine pH during systemic acidosis and fractional excretion of bicarbonate (FEHCO3-) during the condition of normal plasma bicarbonate concentration revealed the disease to be type IV RTA. With elevated plasma renin activity and aldosterone level, the diagnosis of type IV-4 RTA (pseudohypoaldosteronism) was made. Type IV RTA is the most common form of RTA, therefore it is recommended that young infants with suspected RTA should be checked for serum aldosterone level first.
Acidosis ; Acidosis, Renal Tubular* ; Aldosterone ; Diagnosis ; Humans ; Hydrogen-Ion Concentration ; Hypoaldosteronism ; Infant ; Male ; Plasma ; Pseudohypoaldosteronism ; Renin

Hyperkalemia is often detected in young infants, particularly in association with acute pyelonephritis or a urinary tract anomaly. Cases of hyperkalemia in this population may also be due to transient pseudohypoaldosteronism, or immaturity of renal tubules in handling potassium excretion. Symptoms of hyperkalemia are non-specific, but are predominantly related to skeletal or cardiac muscle dysfunction, and can be fatal. Therefore, treatment has to be initiated immediately. Administration of fludrocortisone for hyperkalemia is appropriate in cases with hypoaldosteronism, but is challenging in young infants with hyperkalemia due to renal tubular immaturity, without pseudohypoaldosteronism. We report the case of a 25-day-old male presenting with persistent hyperkalemia with normal serum aldosterone, who was admitted with a first episode of pyelonephritis and unilateral high-grade vesicoureteral reflux. The patient was treated successfully with fludrocortisone.
Aldosterone* ; Fludrocortisone ; Humans ; Hyperkalemia* ; Hypoaldosteronism ; Infant ; Male ; Myocardium ; Potassium ; Pseudohypoaldosteronism ; Pyelonephritis* ; Urinary Tract ; Vesico-Ureteral Reflux

A 49-year-old man with liver cirrhosis and hypertension was found to have hyperkalemia out of a degree of renal insufficiency and metabolic acidosis with low to normal anion gap, aggravated by volume contraction with diarrhea and medications (captopril, spironolactone and atenolol) interfering with potassium homeostasis. Plasma renin activity and serum aldosterone levels of this patient on a regular diet after discontinuation of medications were very low compared to those of five other cirrhotic patients with normokalemia as controls. Also, the renin-aldosterone stimulation testing on this patient performed by sodium restricted diet and furosemide, upright position and by angiotensin converting enzyme inhibition (captopril, 50 mg) showed the blunted renin and aldosterone responses to each of these stimuli, almost no changes from baseline renin and aldosterone levels, it was concluded that the underlying defect responsible for hyperkalemia in this case was hyporeninemic hypoaldosteronism and this was aggravated by other factors or drugs affecting potassium homeostasis.
Aldosterone/blood ; Captopril/pharmacology ; Furosemide/pharmacology ; Humans ; Hyperkalemia/*etiology ; Hypertension/*complications ; Hypoaldosteronism/*complications ; Liver Cirrhosis/*complications ; Male ; Middle Aged ; Renin/blood

This study was aimed at investigating the mechanisms of clinically important overt hyperkalemia in diabetes mellitus with underlying hyporeninemic hypoaldosteronism known as a classic model of the syndrome of hyporeninemic hypoaldosteronism (SHH). Rats (Sprague-Dawley, male) were streptozotocin-treated (60 mg/kg, ip) and used after 60 days. Rats with plasma glucose levels higher than 300 mg/dL (mean +/- SEM, 423 +/- 20 mg/dL, n = 8) were selected as the diabetic group. Age-matched normal rats served as control (mean plasma glucose, 88 +/- 2, mg/dL, n = 8). Serum potassium concentrations and osmolalities as well as serum creatinine levels were significantly higher in the diabetic than in the control group (5.07 +/- 0.09 vs. 4.68 +/- 0.11 mEq/L; 330 +/- 14 vs 290 +/- 3 mOsm/L; 0.40 +/- 0.03 vs 0.31 +/- 0.02 mg/dL, p < 0.05). Plasma renin activity (PRA) in the diabetic group was significantly lower than that in the control group (6.0 +/- 1.0 vs 12.1 +/- 1.1 ng Al/ml/h, p < 0.001). Plasma aldosterone concentration (PAC) was also significantly lower in the former than in the latter (368 +/- 30 vs 761 +/- 57 pg/ml, p < 0.001). Renomegaly, abnormal distal tubular cells with few organelles, and increased lipid droplets with pyknotic nucleus in zona glomerulosa of the adrenal glands were noted in the diabetic group. In conclusion, multifactorial causes including insulinopenia, hyperosmolality, elevated serum creatinine level and hypoaldosteronism with possible contribution of altered distal tubular response to aldosterone may have interacted to develop hyperkalemia in these diabetic rats.
Animals ; Diabetes Mellitus, Experimental/blood/*complications/pathology ; Disease Models, Animal ; Hyperkalemia/*complications ; Hypoaldosteronism/*complications ; Kidney Tubules, Distal/ultrastructure ; Male ; Rats ; Rats, Sprague-Dawley ; Reference Values ; Zona Glomerulosa/ultrastructure

After renal transplantation, we are more likely to encounter hyperkalemia rather than hypokalemia. We report a case of kidney transplantation recipient with hypokalemia and hypertension secondary to primary aldosteronism. A 48 year-old woman was presented with fatigue and weight loss that had lasted for 3 months. She was diagnosed as autosomal dominant polycystic kidney disease that ultimately progressed to end-stage renal disease. She was operated for renal transplantation before 6 months. She had hypokalemia and hypertension at that time. The ratio of plasma aldosterone over plasma renin activity was 851.7. The computed tomography (CT) revealed 2.4x1.7 cm sized adrenal mass on the right side. The pre-transplantation CT also showed that there had been adrenal mass in the same location even before the transplantation. Right adrenalectomy was performed. After she got discharged, she was again presented with nausea and vomiting. She developed hyperkalemia and was diagnosed as hyporeninemic hypoaldosteronism. She was prescribed with fludrocortisones and recovered from the disease, and resumed the state of normokalemia and normotension.
Adrenalectomy ; Aldosterone ; Fatigue ; Female ; Humans ; Hyperaldosteronism ; Hyperkalemia ; Hypertension ; Hypoaldosteronism ; Hypokalemia ; Kidney Failure, Chronic ; Kidney Transplantation ; Nausea ; Plasma ; Polycystic Kidney, Autosomal Dominant ; Renin ; Transplants ; Vomiting ; Weight Loss

Primary aldosteronism is due to either a unilateral adrenal adenoma or bilateral hyperplasia of the adrenal cortex in most cases. A unilateral adrenalectomy in hypertensive and hypokalemic patients, with a well-documented adrenal adenoma, is usually followed by the correction of hypokalemia in all subjects, with the cure of hypertension in 60 to 87% of patients. Here, a unique case, in which a unilateral adrenalectomy for the removal of an adrenal adenoma was followed by severe hyperkalemia, low levels of plasma renin activity and serum aldosterone, suggestive of chronic suppression of the renin-aldosterone axis, is reported. In a follow-up Lasix stimulation test on the 70th day after surgery, the suppression of the renin-aldosterone axis was resolved, indicating the suppression was transient. Patients undergoing a unilateral adrenalectomy for an aldosterone-producing adenoma should be closely followed up to avoid severe hyperkalemia.
Adenoma* ; Adrenal Cortex ; Adrenalectomy ; Aldosterone ; Axis, Cervical Vertebra ; Follow-Up Studies ; Furosemide ; Humans ; Hyperaldosteronism ; Hyperkalemia ; Hyperplasia ; Hypertension ; Hypoaldosteronism* ; Hypokalemia ; Plasma ; Renin

Potassium balance and serum potassium level are maintained until very late in chronic kidney disease (CKD), mainly because of an increase in renal and colonic excretion. Hyperkalemia may develop earlier in the course of CKD in patients with hyporeninemic hypoaldosteronism. Hyperkalemia in CKD patients may occur in association with excess dietary potassium intake, constipation or prolonged fasting. It may also be seen with the use of potassium-sparing diuretics, angiotensin converting enzyme inhibitors, angiotensin receptor blockers, and non-steroidal anti-inflammatory drugs. If suspected, pseudohyperkalemia should be excluded to avoid unnessary treatments. Acute treament of hyperkalemia in marked or symptomatic hyperkalemia, particularly in the presence of electrocardiographic changes includes combinations of intravenous calcium gluconate and infusions of glucose and insulin with or without bicarbonate. In patients with kidney failure, dialysis may be required. Either asymptomatic and mild hyperkalemia or chronic hyperkalemia in CKD patients can be treated by potassium restriction, a loop diuretic at high doses, and cation exchange resin.
Angiotensin Receptor Antagonists ; Angiotensin-Converting Enzyme Inhibitors ; Calcium Gluconate ; Colon ; Constipation ; Dialysis ; Diuretics ; Electrocardiography ; Fasting ; Glucose ; Humans ; Hyperkalemia* ; Hypoaldosteronism ; Insulin ; Potassium ; Potassium, Dietary ; Renal Insufficiency ; Renal Insufficiency, Chronic*