Wilson-Konovalov's disease (DWK) is a polysyndrome hereditary disease associated with excessive accumulation of copper due to the delay in its removal from the body. The condition of the reproductive system in patients with DWK is not well understood. It is proved that copper is a microelement necessary for the synthesis of estrogens, the secretion of prostaglandins in the endometrium. According to the results of the study, in reproductive age patients with DWK who do not receive adequate therapy, the reproductive function is impaired. Often amenorrhea, spontaneous abortions, infertility are noted. Violation of the reproductive function in this disease is associated primarily with liver failure and toxicity of copper. In the absence of treatment, copper, not bound by ceruloplasmin, penetrates from the plasma into the tissues, disrupting the function of the ovaries by reducing aromatase activity. At the heart of these disorders is the toxic effect of copper on the ovaries. Pregnancy in DWK is not contraindicated in the absence of liver failure and portal hypertension. During pregnancy, the negative balance of copper, in addition to adherence to diet and drug therapy, is supported by additional consumption of copper for the construction of fetal tissues, and subsequently for lactation. The onset of pregnancy is desirable only after the normalization of transaminase activity and the transition to the phase of maintenance therapy. Treatment should not stop; The risk of withdrawal of treatment during pregnancy is greater than the risk of continuing it. To date, sufficient experience has been accumulated in the treatment of d-penicillamine, trientine, zinc preparations during pregnancy and the absence of teratogenic action. The article presents a clinical case of a favorable outcome of pregnancy in (DWK). This example shows that the use of chelates and zinc salts in patients with (DWK) is associated with a positive pregnancy outcome for the mother and fetus.
Keywords: Wilson-Konovalov’s disease; pregnancy; reproductive system.