Rotor syndrome
Contents:
Description:
The syndrome of the Rotor (Rotor syndrome) — a hereditary pigmental hepatosis, reminds an easy form of syndrome Dabina — Johnson — defect of excretion of bilirubin is less expressed. Differences: lack of the 2nd peak on a curve of allocation of a bromsulfalein, is found a gall bladder at the cholecystography, there is no formation of a dark pigment in hepatic cells.
The direct hyperbilirubinemia without disturbance of activity of liver enzymes is characteristic of a hereditary syndrome of the Rotor. The Rotor syndrome, as a rule, arises at children's age, is inherited on autosomal recessively type. Find symptoms of fatty dystrophy in hepatocytes.
Rotor Syndrome symptoms:
It is shown by intermittent jaundice. The disease comes to light since the childhood, bilirubin accrues, the content of coproporphyrin in urine increases, is late бромсульфалеин, the gall bladder in this case is contrasted. At the majority the disease proceeds asymptomatically.
Rotor Syndrome reasons:
Has a hereditary etiology, on autosomal recessively type.
Treatment of the Syndrome of the Rotor:
* removal of the conjugated bilirubin (a profound diuresis, absorbent carbon as bilirubin adsorbent in intestines);
* binding of already circulating bilirubin in blood (administration of albumine in a dose of 1 g/kg of weight within 1 hour). Administration of albumine before zamenny hemotransfusion is especially reasonable;
* destruction of the bilirubin fixed in fabrics receptors, peripheral receptors which can connect new portions of bilirubin thereby are released, its penetration through a blood-brain barrier is prevented. It is reached by means of phototherapy. The maximum effect is observed at the wavelength of 450 nanometers. Lamps with blue light are more effective, however they complicate observation of the child's skin. A photosource place at distance 40 — 45 cm over a body (to carry out the procedure only in the couveuse at control of temperature). Eyes of the child need to be protected. Photodegradation of bilirubin is strengthened by Riboflavinum which is even in intracellular concentration chromophore. The necessary duration of phototherapy decreases also at addition of a holestiramin though this drug is less physiologic, than Riboflavinum. The phototherapy is much more effective at simultaneous holding sessions of an oxygenobarotherapy as oxygen strengthens bilirubin decomposition;
* the aspiration to avoid provocative factors (an infection, an overload), drugs — competitors of a glyukoronirovaniye or forcing out bilirubin from communication with albumine (oral contraceptives, streptocides, heparin, salicylates). It is necessary to avoid the states which are followed by a hyperpermeability of a blood-brain barrier (acidosis);
* use of sufficient water loading (prevention of a syndrome of a pachycholia);
* Insolation is contraindicated
* A diet with restriction of the refractory fats and products containing preservatives. Group B vitamins.
* Cholagogue means are recommended.
* Sanitation of the chronic centers of an infection and treatment of the available pathology of biliary tract.
* in critical cases — exchange hemotransfusion.
