Type I renal tubular acidosis
Introduction
Introduction to type I renal tubular acidosis Type I renal tubular acidosis is a defect in distal renal tubular function. It can not establish an effective pH gradient between renal tubular fluid and pericardial fluid. Hydrogen secretion and ammonia production are reduced, so that hydrogen ions are retained in the body, resulting in Acidosis. Family sex is more common in adult women, and the infant type is short-lived, with little recurrence after improvement. basic knowledge The proportion of illness: 0.0002%-0.0003% Susceptible people: more common in adult women Mode of infection: non-infectious Complications: rickets, fibrous osteitis, renal insufficiency, renal failure
Cause
Causes of type I renal tubular acidosis
Genetic, autoimmune diseases (45%):
Primary, family and sporadic and may be associated with certain genetic diseases such as osteopetrosis, hepatolenticular degeneration, carbonic anhydrase deficiency; primary hypergammaglobulinemia, systemic lupus erythematosus, dry synthesis Sign, Hashimoto's thyroiditis, cirrhosis.
Calcium metabolism disorders, renal interstitial disorders (37%):
Idiopathic urinary hypercalcemia, hyperparathyroidism, excessive vitamin D, etc.; obstructive nephropathy, renal transplant rejection, sponge kidney, painkiller nephropathy, sickle cell disease.
Drug-induced: amphotericin B, lithium, toluene and ampicillin.
Prevention
Type I renal tubular acidosis prevention
If the primary DRTA can be diagnosed and treated early, the HCO-3 can be maintained at a normal level, the acidosis can be completely corrected, the clinical symptoms can disappear, the growth and development reach the normal standard within 2 years, and the prognosis is good, such as the kidney at the time of diagnosis. Calcification, leaving more kidney dysfunction.
Complication
Complications of type I renal tubular acidosis Complications rickets fibrous osteitis renal insufficiency renal failure
Clinically, rickets and fibrotic osteitis may occur, and renal insufficiency or renal failure may occur in the advanced stage.
Symptom
Type I renal tubular acidosis symptoms Common symptoms Nausea fatigue, polyuria, nausea and vomiting, palpitations, shortness of breath, dehydration, arrhythmia, osteoporosis, bone pain
First, metabolic acidosis: may have anorexia, nausea, vomiting, palpitations, shortness of breath, fatigue and other symptoms, infant type growth retardation.
Second, electrolyte imbalance:
1 distal renal tubular secretion H + dysfunction, urine NH4 + and titratable acid discharge decreased, urine pH can not be reduced to 6.0, urinary potassium, sodium, calcium excretion increased, patients often have polydipsia, polyuria, and water loss, etc. symptom.
2 increased urinary sodium excretion, decreased blood sodium, in order to increase sodium absorption, increase aldosterone secretion, exacerbate potassium excretion from the urine, prone to low sodium, hypokalemia, patients often have muscle weakness and soft palate, severely Affects breathing and causes arrhythmia.
3 increased urinary calcium, often caused by decreased blood calcium, can cause hyperparathyroidism, promote the dissolution rate of bone, and the presence of acidosis, alkaline minerals in the bone are absorbed, aggravating bone decalcification, often expressed as Fibrous osteitis, osteoporosis, and even pathological fractures, increased urinary calcium excretion, renal tubular secretion H + function is low, the formation of calcium stones in the kidney is easy to form, if caused by obstruction, will increase the degree of renal tubular acidosis.
Examine
Examination of type I renal tubular acidosis
First, the urine pH value is above 6.0.
Second, there is low blood sodium, hypokalemia and hypocalcemia, blood chlorine is significantly increased.
Third, X-ray examination found osteoporosis, pathological fractures or urinary tract stones.
Fourth, ammonium chloride load test.
Five, blood, urine, carbon dioxide tension determination.
Diagnosis
Diagnosis and differentiation of type I renal tubular acidosis
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
The differential diagnosis is mainly related to chronic renal failure, severe dehydration acidosis, pituitary dwarfism and renal diabetes insipidus.
Identification of type I renal tubular acidosis and type II renal tubular acidosis
Type I renal tubular acidosis is a defect in distal renal tubular function. It can not establish an effective pH gradient between renal tubular fluid and pericardial fluid. Hydrogen secretion and ammonia production are reduced, so that hydrogen ions are retained in the body, resulting in Acidosis, family sex is more common in adult women, infant type is short-lived, rarely relapse after improvement.
Type II renal tubular acidosis is a phenomenon in which the proximal renal tubular reabsorption of sodium carbonate is reduced, resulting in a decrease in the blood, which is characterized by high chloride acidosis, which is reduced by the proximal tubule reabsorption of sodium carbonate. If the sodium is increased, if the absorption threshold is exceeded, the urinary sodium carbonate will increase and the urine pH will increase. If the sodium carbonate in the blood drops to a certain extent, the renal tubule can completely absorb the sodium carbonate in the glomerular filtrate, then the urine The acidity can be restored, and the urine pH can be lowered. In addition, the proximal renal tubules can also reduce glucose, phosphate, uric acid, and amino acid reabsorption, and the performance is Fanconi syndrome.
