ABSTRACT
BACKGROUND: Type II pseudohypoaldosteronism is a rare tubulopathy defined by abnormal renal potassium excretion. CASE REPORT: A 12 1/2 year-old girl, was admitted for dwarfism. Her parents were not consanguineous and her 5 living sibs were normal. At admission, she had moderate hypertension: systolic 130-150 mmHg; diastolic 80-100 mmHg and no pubertal development. LABORATORY DATA: pH (arterial): 7.34; bicarbonates: 18-20 mEq/l; chloride: 112-120 mEq/l; potassium: 5.6-7 mEq/l; aldosterone: 200-700 pg/ml (N < 60); plasma renin activity: 0.4 ng/ml/hr (N 2.2 +/- 0.2). The bone maturation was 8 1/2 years. All the other renal function tests were normal. The titratable acidity was 22 mEq/day (N 20-40) and the ammonia excretion 15.2 mEq/l (N 44-61). The fractional excretion of potassium was 6.5% (N 11.8 +/- 1.9). This girl was given polystyrene sulfonate resin followed by hydrochlorothiazide (1 to 3 mg/kg/day). There was a subsequent improvement in all data, a growth spurt and pubertal development. CONCLUSION: This is the fifth case of type II pseudohypoaldosteronism reported in childhood and the first one with hypertension. The beneficial effect of hydrochlorothiazide is underlined.
Subject(s)
Acidosis, Renal Tubular/drug therapy , Dwarfism/drug therapy , Hydrochlorothiazide/therapeutic use , Hyperkalemia/drug therapy , Hypertension/drug therapy , Pseudohypoaldosteronism/complications , Acidosis, Renal Tubular/etiology , Child , Dwarfism/etiology , Female , Humans , Hyperkalemia/etiology , Hypertension/etiologyABSTRACT
Ultrafiltrates from uremic patients can induce, in vitro, strong modifications of beating frequency of auricular fragments of the embryonic chick heart. According to concentration, effects increase from tachycardia to cessation of cardiac beats. Complete reversion is always observed after washing with normal medium. Only fraction 7c of middle molecules exhibits this cardiotoxicity, other fractions are without effect, even in high concentration. Uremic middle molecules strongly modify cardiac beats in vitro.
Subject(s)
Heart/drug effects , Toxins, Biological/toxicity , Uremia/blood , Animals , Chick Embryo , Dose-Response Relationship, Drug , Heart Rate/drug effects , Humans , Renal DialysisABSTRACT
Middle molecules can act on several levels of uremic disease. So, this work is a multidisciplinary one with the participation of nephrologists, hematologists and also pharmacologists, chemists and biophysicians. We present here our results on four different toxic effects found with middle molecules: the energy dependant phenomena, the platelet aggregability, the cardiotoxic, and the neurotoxic effects. Middle molecules were isolated from urine of normal subjects and blood of patients with chronic renal insufficiency and treated by hemodialysis. Separation was done according to Furst's procedure.
Subject(s)
Platelet Aggregation/drug effects , Toxins, Biological/physiology , Animals , Biological Assay , Energy Metabolism/drug effects , Heart/drug effects , Humans , Neurotoxins , Ranidae , Sciatic Nerve/drug effects , Sciatic Nerve/physiology , Toxins, Biological/pharmacology , Uremia/physiopathologyABSTRACT
In 20 patients suffering from chronic renal failure treated by hemodialysis (CRFH) the pure pancreatic juice (PPJ) collected via endoscopic cannulation of the papilla was studied. PPJ was collected during 20 minutes in 1 minute samples. Secretin and pancreozymin (CCK-PZ) were given as boluses respectively at the beginning and at the 10th minute of the test. Volumes, total protein and bicarbonate concentrations, total protein and bicarbonate outputs, Lactoferrin (LF) levels were measured. The results observed in patients were compared to those of ten subjects free of any organic disease. The volume of PPJ was significantly more important in CRFH than in controls. Bicarbonate and protein concentrations were not significantly modified. Protein output was not significantly more important in CRFH at the exception of the 12th sample. Bicarbonate output was not significantly increased at the exception of the 9th and the 11th samples. LF level was more than 0.03% of total proteins in 4 CRFH.
Subject(s)
Kidney Failure, Chronic/physiopathology , Pancreas/metabolism , Renal Dialysis , Adult , Bicarbonates/analysis , Cholecystokinin , Female , Humans , Kidney Failure, Chronic/therapy , Lactoferrin/analysis , Male , Middle Aged , Pancreatic Juice/analysis , Proteins/analysis , SecretinABSTRACT
"Uremic middle molecules" are isolated from the urine of normal subjects and the blood in chronic renal insufficiency treated by hemodialysis. Their action is tested in vitro on platelets aggregation induced by ADP. Some fractions of these middle molecules (fractions 7b, 7c and 7 "fg") are without any effect. The 7 "de" fractions give an inhibition of platelets aggregation. The percentage variations of first phase inhibition as a function of the middle molecules concentrations is studied.
