pituitrin and Acidosis--Renal-Tubular

Topics: Acidosis, Renal Tubular; Aldosterone; Ascites; Diuretics; Hepatic Encephalopathy; Humans; Hypokalemia; Kidney Failure, Chronic; Kidney Transplantation; Kidney Tubules, Distal; Liver Cirrhosis; Liver Transplantation; Sodium; Transplantation, Homologous; Uremia; Vasopressins; Water-Electrolyte Imbalance

Topics: Acidosis, Renal Tubular; Electrolytes; Humans; Kidney Diseases; Kidney Failure, Chronic; Magnesium; Muscular Diseases; Neurologic Manifestations; Vasopressins

A 19-year-old girl presented at our emergency room with hypokalemic periodic paralysis. She had a thyrotoxic goiter and had experienced three paralytic attacks during the previous 2 years on occasions when she stopped taking antithyroid drugs. In addition to thyrotoxic periodic paralysis (TPP), she had metabolic acidosis, urinary potassium loss, polyuria and polydipsia. Her reduced ability to acidify urine during spontaneous metabolic acidosis was confirmed by detection of coexisting distal renal tubular acidosis (RTA). The polyuria and polydipsia were caused by nephrogenic diabetes insipidus, which was diagnosed using the water deprivation test and vasopressin administration. Her recurrent and frequent paralytic attacks may have been the combined effects of thyrotoxicosis and RTA. Although the paralytic attack did not recur after improving the thyroid function, mild acidosis and nephrogenic DI have been remained subsequently. Patients with TPP, especially females with atypical metabolic features, should be investigated for possible precipitating factors.

Topics: Acidosis, Renal Tubular; Adult; Antithyroid Agents; Diabetes Insipidus, Nephrogenic; Female; Goiter; Humans; Hypokalemic Periodic Paralysis; Medication Adherence; Polyuria; Propylthiouracil; Recurrence; Thyrotoxicosis; Vasopressins; Water Deprivation

In five members of three generations in a family studied in 1972, 3 nephrological disorders occurred concurrently: distal renal tubular acidosis (dRTA), polycystic kidney and nephrogenic diabetes insipidus (with the exception of a five-year-old child in whom polycystic kidney was not detectable--yet?). Chromosoma studies revealed an increased rate of the occurrence of variations. The youngest patient was reinvestigated in 1993; the other four affected members of the family were already not alive. 7 offsprings in two generations of the 3 healthy members of the third "patient generation" were healthy. The autosomal dominant way of inheritance characteristic to both dRTA and polycystic kidney disease was obvious in this family. On the other hand the same degree of the concentrating defect found both in the patients of the familial dRTA and in 11 control patients with non familial (acquired) dRTA suggested that the nephrogenic diabetes insipidus as an acquired disorder was associated with the two congenital abnormalities. The clinical picture of the combined disease was dominated by the symptoms of polydipsia and polyuria. The vasopressin resistance with a variation in the degree interindividually seemed to be responsible for the nephrogenic diabetes insipidus. Functional insufficiency of the loop of Henle was excluded on the basis of normal responses to a "loop diuretic".

Topics: Acidosis, Renal Tubular; Adolescent; Adult; Aged; Child; Child, Preschool; Diabetes Insipidus, Nephrogenic; Drinking; Female; Humans; Male; Middle Aged; Nephrocalcinosis; Pedigree; Polycystic Kidney, Autosomal Dominant; Polyuria; Radiography; Vasopressins

We observed idiopathic light-chain proteinuria in a patient with multiple abnormalities of proximal-tubule transport mechanisms (Fanconi syndrome), nephrogenic diabetes insipidus, and distal renal tubular acidosis. Seventeen of the 19 urinary amino acid levels measured were elevated. Uric acid and phosphate clearances were greater than 60 per cent and 50 per cent, respectively, of the simultaneous inulin clearance. When water deprivation was coupled with vasopressin administration, the maximum urinary concentration observed was 384 mOsm per kilogram of water. During ammonium-chloride loading, the level of hydrogen-ion concentration in the urine remained less than 100 times that in the blood. Kappa light-chain excretion was 149 mg per 24 hours. It appears that the concurrence of proximal tubular dysfunction, distal tubular dysfunction and light-chain proteinuria represents a distinct syndrome, which we call "combined light-chain nephropathy." Available evidence indicates that excessive light-chain production with subsequent filtration, reabsorption and catabolism, causes the complex tubular dysfunctions observed.

Topics: Acidosis, Renal Tubular; Ammonium Chloride; Bence Jones Protein; Diabetes Insipidus; Fanconi Syndrome; Female; Follow-Up Studies; Humans; Immunoglobulin kappa-Chains; Immunoglobulin Light Chains; Kidney Concentrating Ability; Kidney Diseases; Kidney Tubules; Kidney Tubules, Distal; Middle Aged; Proteinuria; Renal Aminoacidurias; Syndrome; Vasopressins

Renal function studies were done in five children with infantile polycystic disease (IPCD)of kidneys and liver and in four with congenital hepatic fibrosis (CHF). Glomerular filtration rate was reduced in all IPCD patients and in two of four CHF patients. Urinary concentrating ability following water deprivation and vasopressin administration was impaired in all IPCD patients and in three of four CHF patients. During control period, all patients had asymptomatic metabolic acidosis with total carbon dioxide content less than or equal to 20.5 millimols/liter, and net acid excretion (NAE) was reduced in all but one. Ammonium chloride was administered to seven patients; NAE increased in all, but the increments were subnormal in four. The inability to excrete maximally concentrated urine and an adequate amount of net acid may best be explained by abnormal tubular structure or alterations in medullary architecture secondary to progressive scarring, or both.

Topics: Acidosis, Renal Tubular; Adolescent; Ammonium Chloride; Child; Child, Preschool; Cystic Fibrosis; Female; Glomerular Filtration Rate; Humans; Infant; Kidney Concentrating Ability; Kidney Diseases; Kidney Function Tests; Liver Cirrhosis; Male; Vasopressins; Water Deprivation

Topics: Absorption; Acidosis, Renal Tubular; Adult; Creatinine; Female; gamma-Globulins; Glomerular Filtration Rate; Humans; Hydrogen-Ion Concentration; Hypergammaglobulinemia; Kidney Function Tests; Kidney Tubules; Lupus Erythematosus, Systemic; Male; Middle Aged; Osmolar Concentration; Phosphates; Prognosis; Urine; Vasopressins

Investigation of a patient with hypercalcaemia, hypophosphataemia, and nephrocalcinosis failed to lead to a clear diagnosis. Neither primary hyperparathyroidism nor primary incomplete renal tubular acidosis could explain all the biochemical features, and it seems that more than one fundamental abnormality may have been present.

Topics: Acidosis; Acidosis, Renal Tubular; Adolescent; Ammonium Chloride; Calcium; Calcium, Dietary; Diagnosis, Differential; Humans; Hydrogen-Ion Concentration; Hypercalcemia; Hyperparathyroidism; Male; Nephrocalcinosis; Osmolar Concentration; Phosphates; Urine; Vasopressins

Topics: Acidosis, Renal Tubular; Ammonium Chloride; Bicarbonates; Calcium; Child; Erythrocytes; Female; Growth Hormone; Humans; Kidney; Kidney Tubules; Magnesium; Male; Nephrocalcinosis; Parathyroid Hormone; Vasopressins

Topics: 17-Hydroxycorticosteroids; Acetazolamide; Acidosis, Renal Tubular; Ammonium Chloride; Bicarbonates; Blood Urea Nitrogen; Chlorides; Creatinine; Extracellular Space; Humans; Hydrogen-Ion Concentration; Kidney Function Tests; Male; Middle Aged; Potassium; Renin; Sodium; Vasopressins

Topics: Acidosis; Acidosis, Renal Tubular; Amino Acids; Humans; Kidney Diseases; Polyuria; Vasopressins