TUBULOPATYI (Latin tubula a tube + Greek pathos suffering, a disease) — group of the diseases which are characterized by permanent disorders of function of renal tubules. At the heart of T. disturbance of transport in renal tubules of various substances lies.
T. happen hereditary (primary) and acquired (secondary). At primary T. membrane transport in renal tubules is broken. Causes of infringement can be various: structural changes of membrane carrier proteins, insufficiency of the enzymes providing membrane transport, changes of sensitivity of receptors of cells of a canalicular epithelium to the regulating effect of hormones and other biologically active agents, changes of the general structure of membranes of cells (dysplasia).
Secondary T. develop owing to damage of transport systems of renal tubules at diseases of a metabolism, at defeat of tubules of kidneys chemical substances and pharmaceuticals, at inflammatory processes in kidneys. At inflammatory diseases of kidneys of disturbance of transport in renal tubules happen temporary (if does not form hron. a renal failure) therefore it is not absolutely lawful to carry them to a tube of l to opatiya.
T. classify by localization of defect and conducting a wedge, to a sign. On localization of defect allocate T. with preferential defeat of proximal gyrose tubules, distal gyrose tubules and collective channels, tubulopatiya with damage of all
canalicular device. K T. with defeat of proximal gyrose tubules carry: de Tony's syndrome — Debra — Fankoni (see De Tony — Debra — Fankoni a syndrome), a glycosuria (see), phosphate diabetes (see), a cystinuria (see), an iminogli-tsinuriya and a glycinuria (see Glycine a riya), Hartnup's disease (see Hart-nupa a disease), renal tubular acidosis like II (see Laytvud — Albright a syndrome), and also secondary defeats at a cystinosis (see), Lowe's syndrome (see Lowe a syndrome), a tyrosinosis (see), a galactosemia (see), glycogenoses (see), fruktozemiya (see), poisonings with salts of heavy metals, lysol, cresol, tetracycline, etc., at Wilson's disease — Konovalova (see Gepato-tserebraljnaya dystrophy), primary giperpar an athyreosis (see), hypophosphatasias (see), Gee's diseases (see), Alport's syndrome (see Aljport a syndrome), primary hyperoxaluria (see Oksaluriya), a diabetes mellitus (see a diabetes mellitus), xanthinurias (see Xanthine). Defeat of distal gyrose tubules and collective channels is observed at a renal not diabetes mellitus (see Diabetes not sugar), renal tubular acidosis like I (see Laytvud — Albright a syndrome), psevdogipoaljd an oster oniz-
me (see), and also at pyelonephritis (see). Damage of all canalicular device is noted at T., developed as a result hron. a renal failure (see) and Fankoni's nefronoftiza (see Fankoni nefronoftiz).
Similarity of the main symptoms allows to combine T, various on genesis. in three groups. The leading sign of one of them are rakhitopodobny changes of a skeleton, another — a polyuria, the third — a nephrolithiasis.
T. with rakhitopodobny changes of a skeleton (see the Osteopathy nephrogenic) include phosphate diabetes, de Tony's syndrome — Debra — Fankoni, renal tubular acidosis, and also vitamin-B-dependent rickets (see), a hypophosphatasia (see), a Gee's disease, psevdogipopar an athyreosis (see). General signs of this group T. the growth inhibition, the deformation of a bone skeleton appearing at the age of 1 — 2 years, a hypomyotonia, ostealgias and muscles, a calciuria (see the Nephrocalcinosis) and a fosfaturiya are (see); on roentgenograms rakhitopodobny changes of bones are noted.
K T., the main symptom to-rykh is the polyuria (see), a renal glycosuria (see Diabetes renal), renal not sugar (vazopressinrezistentny) diabetes, a pseudo-hypoaldosteronism, and also damages of kidneys at a nefronoftiza of a fankona, pyelonephritis, a cystinosis belong * a tyrosinosis, hron. renal failure.
KT., the main manifestation to-rykh — a nephrolithiasis (see the Nephrolithiasis), the caused disturbances of transport of amino acids in kidneys at a tsistp-nuriya, a glycinuria, an iminoglitspnu-riya belong hereditarily, Hartnup's diseases and secondary changes at an oxarod (see), a secondary hyperoxaluria (see Oksaluriya), a xanthinuria, Lesh's syndrome — Naykhana (see Gout).
Bibliography: Badalyan JI. Lake, Thabo lean V. A. and Veltishchev Yu. E. Hereditary diseases at children, page 257, M., 1971; Diseases of kidneys, under the editorship of G. Maj-drakova and N. Popov, lane with bolg., page 268, Sofia, 1973; Ignatova M. S. and Veltishchev Yu. E. Children's nephrology, page 283, L., 1982; Shilov A. V. and Novikov P. V. Differential diagnosis of rakhitopodobny diseases at children, Pediatrics, No. 9, page 65, 1979; Woolf L. I. Renal tubular dysfunction, Springfield, 1966, bibliogr.
And. H. Kalmykova, V. P. Lebedev.