Unlike most other inborn errors of metabolism, which require advanced and expensive diagnostic techniques and complex drug and dietary management (often not feasible in developing countries), the renal tubular acidoses may be detected and treated both easily and cheaply. Diagnostic confusion is possible as this series demonstrates due to the protean clinical manifestations. Three recent cases from Port Moresby General Hospital are described and appropriate investigations and treatment discussed.
Acidosis, Renal Tubular - diagnosis ; Acidosis, Renal Tubular - metabolism ; Child, Preschool ; Female ; Humans, Infant

To evaluate urinary acidification defect and its contribution to metabolic acidosis (MA) during hemorrhagic fever with renal syndrome (HFRS), we serially analyzed acid-base balance and urinary acidification indices in 10 HFRS patients. Data of the patients were compared with those of 8 normal volunteers (NC). MA was observed in 6 of 8 patients in the oliguric phase, 5 of 7 in the early diuretic phase, 8 of 10 in the late diuretic phase and 2 of 9 in the convalescent phase. HFRS patients with MA had a higher plasma anion gap in the oliguric and early diuretic phases than NC and a higher plasma Cl/Na ratio in the late diuretic phase than NC. As compared with acid-loaded NC, HFRS patients had a higher urine pH in the oliguric, early diuretic and late diuretic phases, a higher urine anion gap (UAG) in the oliguric and early diuretic phases and a lower urinary NH4+ excretory rate in the oliguric, early diuretic and late diuretic phases. Overt distal acidification defect was observed in 6 of 8 patients in the oliguric phase, 3 of 7 in the early diuretic phase, 5 of 10 in the late diuretic phase and none of 9 in the convalescent phase. None of the convalescent patients had latent acidification defect. In conclusion, urinary acidification defect is marked in the oliguric and diuretic phases of severe HFRS and may play a role in the development of a high anion gap (AG) metabolic acidosis in the earlier phase and hyperchloremic MA in the later phase, but rapidly recovers in the convalescent phase.
Acidosis, Renal Tubular/urine ; Acidosis, Renal Tubular/metabolism ; Hemorrhagic Fever with Renal Syndrome/urine ; Hemorrhagic Fever with Renal Syndrome/metabolism ; Human

Primary hyperparathyroidism is a generalezed disorder of calcium, phosphorus and bone metabolism due to an increased secretion of parathyroid hormone. Single parathyroid adenoma is the most common cause of primary hyperparathyroidism. Because parathyroid hormone has been proposed as an important inhibitor of renal bicarbonate reabsorption of proximal tubule, proximal renal tubular acidosis is not rare in primary hyperparaphyroidism. After parathyroid resection, significant hypocalcemia and hypophosphatemia requiring prolonged medical management may develop, termed hungery bone syndrome. We experienced a case of primary hyperparathyroidism associated with proximal renal tubular acidosis, and severe hungry bone syndrome after resection of the adenoma of parathyroid gland.
Acidosis ; Acidosis, Renal Tubular ; Adenoma ; Calcium ; Hyperparathyroidism, Primary ; Hypocalcemia ; Hypophosphatemia ; Kidney Tubules, Proximal ; Metabolism ; Parathyroid Glands ; Parathyroid Hormone ; Parathyroid Neoplasms ; Phosphorus

No abstract available.
Acidosis/*complications/drug therapy ; Acidosis, Renal Tubular/drug therapy/etiology/metabolism/*pathology ; Adult ; Aquaporin 2/analysis ; Biological Markers/analysis ; Biopsy ; Female ; Humans ; Immunohistochemistry ; Kidney Tubules/chemistry/drug effects/*pathology ; Nephrocalcinosis/etiology/pathology ; Proton-Translocating ATPases/analysis ; Sodium Bicarbonate/therapeutic use ; Tomography, X-Ray Computed ; Treatment Outcome

Although renal calcium crystal deposits (nephrocalcinosis) may occur in acute phosphate poisoning as well as type 1 renal tubular acidosis (RTA), hyperphosphatemic hypocalcemia is common in the former while normocalcemic hypokalemia is typical in the latter. Here, as a unique coexistence of these two seperated clinical entities, we report a 30-yr-old woman presenting with carpal spasm related to hypocalcemia (ionized calcium of 1.90 mM/L) due to acute phosphate poisoning after oral sodium phosphate bowel preparation, which resolved rapidly after calcium gluconate intravenously. Subsequently, type 1 RTA due to Sjogren's syndrome was unveiled by sustained hypokalemia (3.3 to 3.4 mEq/L), persistent alkaline urine pH (> 6.0) despite metabolic acidosis, and medullary nephrocalcinosis. Through this case report, the differential points of nephrocalcinosis and electrolyte imbalances between them are discussed, and focused more on diagnostic tests and managements of type 1 RTA.
Acidosis, Renal Tubular/*diagnosis/etiology ; Acute Disease ; Adult ; Antibodies, Antinuclear/blood ; Calcium Gluconate/therapeutic use ; Chronic Disease ; Female ; Humans ; Hydrogen-Ion Concentration ; Hypocalcemia/*chemically induced/complications/drug therapy ; Nephrocalcinosis/complications/*diagnosis/ultrasonography ; Parotid Gland/ultrasonography ; Phosphates/*adverse effects ; Salivary Glands/radionuclide imaging ; Sjogren's Syndrome/complications/*diagnosis/metabolism ; Submandibular Gland/ultrasonography