The article presents information on the main etiology and pathogenesis of Wilson–Konovalov disease, as hepato-lenticular degeneration associated with excessive accumulation of copper in body tissues due to an inherited autosomal recessive defect of a protein that transports copper. A rare case of the onset and course of the disease in a patient who was examined and treated at the Ivano-Frankivsk Regional Children's Clinical Hospital is described. The main focus is on the acute, atypical onset of the disease, in particular the signs of severe intoxication syndrome caused by manifestations of hepatic encephalopathy, steroid-induced diabetes, anemia of complex genesis, coronavirus disease, pyoderma. The difficulties of diagnosing the disease against the background of delayed manifestation of the main pathognomonic manifestations and the absence of reliable signs of the disease are shown. The clinical picture of this clinical case, features of the course are described, the indicators of the main clinical and laboratory research methods are given, the results of instrumental diagnostic methods and genetic examination methods are shown, both in laboratories of Ukraine and abroad, which confirmed the diagnosis of Wilson–Konovalov disease. The treatment of this clinical case is described in detail not only at the local level but also in the main leading research institution of Ukraine «OHMATDYT» and «National Institute of Surgery and Transplantology named after O.O. Shalimov». Recommendations for further observation and treatment of the child at the place of residence after orthotopic transplantation of the left lobe of the liver from a living family donor. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Wilson-Konovalov disease, etiology, pathogenesis, clinical picture, diagnosis, examination, treatment.

Currently, the problem of diagnosing pleuropulmonary blastoma in children remains relevant, because this pathology often runs under the guise of other nosologies, including pneumonia, and is extremely difficult to diagnose. Currently there are no pathognomonic clinical symptoms, specific diagnostic criteria for this disease, almost 60% of patients have nonspecific symptoms. This article presents a rare clinical case of biphasic pulmonary blastoma in a child who was examined and treated at the Ivano-Frankivsk Regional Children's Clinikal Hospital Ivano-Frankivsk Regional Council. The main clinical manifestations, diagnosis and treatment of biphasic blastoma of the lungs in a child based on the observation of a 5-year-old girl who was hospitalized for acute abdominal pain, chest pain and cough. After clinical — laboratory and instrumental methods of examination, right-sided pneumonia and exudative pleurisy were diagnosed. The set of treatment measures did not give the expected positive effect, improving the patient's condition. Additional testing is required for suspected breast cancer. It has been established that the best method for verifying the diagnosis of pulmonary blastoma is multislice computed tomography with intravenous bolus contrast and immunohistochemical examination of the biopsy. The methods of treatment used in this case, including anti-inflammatory and polychemotherapy according to the scheme, the use of radical treatment at the National Cancer Institute in Kyiv — surgery right pneumonectomy with pericardial resection. The child is currently in stable remission for 4.5 years. The described clinical case demonstrates the difficulty of diagnosing lung blastoma in children due to the lack of typical clinical manifestations. The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: children, pneumonia, blastoma, pulmonology, diagnosis, treatment.

Контактна інформація: Алексеєва Юлія Іванівна-к.мед.н., доцент кафедри педіатрії ДВНЗ «Івано-Франківський національний медичний університет» (м. Івано-Франківськ, Україна) Контакта адреса: вул. Галицька,