30 research outputs found
Sliding Luttinger liquid phases
We study systems of coupled spin-gapped and gapless Luttinger liquids. First,
we establish the existence of a sliding Luttinger liquid phase for a system of
weakly coupled parallel quantum wires, with and without disorder. It is shown
that the coupling can {\it stabilize} a Luttinger liquid phase in the presence
of disorder. We then extend our analysis to a system of crossed Luttinger
liquids and establish the stability of a non-Fermi liquid state: the crossed
sliding Luttinger liquid phase (CSLL). In this phase the system exhibits a
finite-temperature, long-wavelength, isotropic electric conductivity that
diverges as a power law in temperature as . This two-dimensional
system has many properties of a true isotropic Luttinger liquid, though at zero
temperature it becomes anisotropic. An extension of this model to a
three-dimensional stack exhibits a much higher in-plane conductivity than the
conductivity in a perpendicular direction.Comment: Revtex, 18 pages, 8 figure
Π’Π°ΡΠ³Π΅ΡΠ½Π°Ρ ΡΠ΅ΡΠ°ΠΏΠΈΡ Ρ Π΅ΡΡΠ²ΠΈΠ·ΠΌΠ° Ρ ΡΠ΅Π±Π΅Π½ΠΊΠ° 9 Π»Π΅Ρ
Reparative giant cell granulomas (RGCG) is a rare benign lesion of the skull bones in children, which is 1.7% of all recorded cases of RGCG. Despite the benign nature of the disease, RGCG often recurs after surgical treatment and can metastasize. One of the rare forms of RGCG is cherubism, characterized by total defeat of the upper and lower jaw, in connection with which it is impossible to carry out radical surgical treatment, especially in children in the period before puberty. In the department of maxillofacial surgery of the Russian Childrensβ Clinical Hospital under ambulatory supervision for 3 years was a child diagnosed with cherubism. During this time, progressive growth of the upper and lower jaw persisted, leading to exorbitalism. After the histological verification of the diagnosis and the approval of the ethical committee, a course of therapy with the drug denosumab was conducted. Histological examination of the tumor denosumab treatment showed a significant response which resulted in disappearance of giant cells. CT before and after the therapy showed an increase in bone density from 65 to 385 HU. The size of tumor nodes reduced, allowing to made contour resection of the mandible. The structure and volume of the upper jaw reached the average value and did not require surgical correction. After the course of the denosumab, there was no growth retardation according to the control X-ray examination of the wrist. inoperable forms of RGCG and cherubism, a complex approach is needed, including a course of denosumab therapy followed by transfer to therapy with alendronic acid. After the therapy, contour resection of excess bone tissue is possible with the purpose of medical and social rehabilitation of such patients.ΠΠΈΠ³Π°Π½ΡΠΎΠΊΠ»Π΅ΡΠΎΡΠ½Π°Ρ ΡΠ΅ΠΏΠ°ΡΠ°ΡΠΈΠ²Π½Π°Ρ Π³ΡΠ°Π½ΡΠ»Π΅ΠΌΠ° (ΠΠΠ Π) β ΡΠ΅Π΄ΠΊΠΎΠ΅ Π΄ΠΎΠ±ΡΠΎΠΊΠ°ΡΠ΅ΡΡΠ²Π΅Π½Π½ΠΎΠ΅ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ΅ΠΉ ΡΠ΅ΡΠ΅ΠΏΠ° Ρ Π΄Π΅ΡΠ΅ΠΉ, ΠΊΠΎΡΠΎΡΠΎΠ΅ ΡΠΎΡΡΠ°Π²Π»ΡΠ΅Ρ 1,7% Π²ΡΠ΅Ρ
ΡΠ΅Π³ΠΈΡΡΡΠΈΡΡΠ΅ΠΌΡΡ
ΡΠ»ΡΡΠ°Π΅Π² ΠΠΠ Π. ΠΠ΅ΡΠΌΠΎΡΡΡ Π½Π° Π΄ΠΎΠ±ΡΠΎΠΊΠ°ΡΠ΅ΡΡΠ²Π΅Π½Π½ΡΠΉ Ρ
Π°ΡΠ°ΠΊΡΠ΅Ρ, ΠΠΠ Π ΡΠ°ΡΡΠΎ ΡΠ΅ΡΠΈΠ΄ΠΈΠ²ΠΈΡΡΠ΅Ρ ΠΏΠΎΡΠ»Π΅ ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΈ ΠΌΠΎΠΆΠ΅Ρ ΠΌΠ΅ΡΠ°ΡΡΠ°Π·ΠΈΡΠΎΠ²Π°ΡΡ. ΠΠ΄Π½Π° ΠΈΠ· ΡΠ΅Π΄ΠΊΠΈΡ
ΡΠΎΡΠΌ ΠΠΠ Π β Ρ
Π΅ΡΡΠ²ΠΈΠ·ΠΌ, Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠ½ΡΠΉ ΡΠΎΡΠ°Π»ΡΠ½ΡΠΌ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΠ΅ΠΌ Π²Π΅ΡΡ
Π½Π΅ΠΉ ΠΈ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠ΅ΠΉ, Π² ΡΠ²ΡΠ·ΠΈ Ρ ΡΠ΅ΠΌ Π½Π΅Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½ΠΈΠ΅ ΡΠ°Π΄ΠΈΠΊΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ, ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎ Ρ Π΄Π΅ΡΠ΅ΠΉ Π² ΠΏΠ΅ΡΠΈΠΎΠ΄ Π΄ΠΎ ΠΏΠΎΠ»ΠΎΠ²ΠΎΠ³ΠΎ ΡΠΎΠ·ΡΠ΅Π²Π°Π½ΠΈΡ. Π ΠΎΡΠ΄Π΅Π»Π΅Π½ΠΈΠΈ ΡΠ΅Π»ΡΡΡΠ½ΠΎ-Π»ΠΈΡΠ΅Π²ΠΎΠΉ Ρ
ΠΈΡΡΡΠ³ΠΈΠΈ Π ΠΎΡΡΠΈΠΉΡΠΊΠΎΠΉ Π΄Π΅ΡΡΠΊΠΎΠΉ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΉ Π±ΠΎΠ»ΡΠ½ΠΈΡΡ ΠΠΈΠ½Π·Π΄ΡΠ°Π²Π° Π ΠΎΡΡΠΈΠΈ ΠΏΠΎΠ΄ Π°ΠΌΠ±ΡΠ»Π°ΡΠΎΡΠ½ΡΠΌ Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΠ΅ΠΌ Π² ΡΠ΅ΡΠ΅Π½ΠΈΠ΅ 3 Π»Π΅Ρ Π½Π°Ρ
ΠΎΠ΄ΠΈΠ»ΡΡ ΡΠ΅Π±Π΅Π½ΠΎΠΊ Ρ Π΄ΠΈΠ°Π³Π½ΠΎΠ·ΠΎΠΌ Β«Ρ
Π΅ΡΡΠ²ΠΈΠ·ΠΌΒ»: Π½Π°Π±Π»ΡΠ΄Π°Π»ΡΡ ΠΏΡΠΎΠ³ΡΠ΅ΡΡΠΈΠ²Π½ΡΠΉ ΡΠΎΡΡ Π²Π΅ΡΡ
Π½Π΅ΠΉ ΠΈ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠ΅ΠΉ, ΠΏΡΠΈΠ²ΠΎΠ΄ΡΡΠΈΠΉ ΠΊ ΡΠΊΠ·ΠΎΠΎΡΠ±ΠΈΡΠΈΠ·ΠΌΡ. ΠΠΎΡΠ»Π΅ Π³ΠΈΡΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠΉ Π²Π΅ΡΠΈΡΠΈΠΊΠ°ΡΠΈΠΈ Π΄ΠΈΠ°Π³Π½ΠΎΠ·Π° ΠΈ ΠΏΠΎΠ»ΡΡΠ΅Π½ΠΈΡ ΠΎΠ΄ΠΎΠ±ΡΠ΅Π½ΠΈΡ ΡΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΠΊΠΎΠΌΠΈΡΠ΅ΡΠ° ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½ ΠΊΡΡΡ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π±. ΠΠΈΡΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ΅ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΎΠΏΡΡ
ΠΎΠ»ΠΈ ΠΏΠΎΡΠ»Π΅ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π± ΠΏΡΠΎΠ΄Π΅ΠΌΠΎΠ½ΡΡΡΠΈΡΠΎΠ²Π°Π»ΠΎ Π²ΡΡΠ°ΠΆΠ΅Π½Π½ΡΠΉ ΠΏΠ°ΡΠΎΠΌΠΎΡΡΠΎΠ·, ΠΎ ΡΠ΅ΠΌ ΡΠ²ΠΈΠ΄Π΅ΡΠ΅Π»ΡΡΡΠ²ΠΎΠ²Π°Π»ΠΈ ΠΌΠ°Π»ΠΎΡΠΈΡΠ»Π΅Π½Π½ΠΎΡΡΡ ΠΈΠ»ΠΈ ΠΈΡΡΠ΅Π·Π½ΠΎΠ²Π΅Π½ΠΈΠ΅ Π³ΠΈΠ³Π°Π½ΡΡΠΊΠΈΡ
ΠΊΠ»Π΅ΡΠΎΠΊ, ΠΎΠ±ΡΠ°Π·ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ½ΠΎΠΉ ΠΈ Π²ΠΎΠ»ΠΎΠΊΠ½ΠΈΡΡΠΎΠΉ ΡΠΊΠ°Π½Π΅ΠΉ. ΠΠΎ Π΄Π°Π½Π½ΡΠΌ ΠΊΠΎΠΌΠΏΡΡΡΠ΅ΡΠ½ΠΎΠΉ ΡΠΎΠΌΠΎΠ³ΡΠ°ΡΠΈΠΈ Π΄ΠΎ ΠΈ ΠΏΠΎΡΠ»Π΅ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ, ΠΎΡΠΌΠ΅ΡΠ΅Π½ΠΎ ΠΏΠΎΠ²ΡΡΠ΅Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ½ΠΎΠΉ ΠΏΠ»ΠΎΡΠ½ΠΎΡΡΠΈ Ρ 65 Π΄ΠΎ 385 HU. ΠΠΎΡΠ»Π΅ ΠΊΡΡΡΠ° ΡΠ΅ΡΠ°ΠΏΠΈΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π± Π·Π°ΠΌΠ΅Π΄Π»Π΅Π½ΠΈΡ ΡΠΎΡΡΠ°, ΠΏΠΎ Π΄Π°Π½Π½ΡΠΌ ΠΊΠΎΠ½ΡΡΠΎΠ»ΡΠ½ΠΎΠ³ΠΎ ΡΠ΅Π½ΡΠ³Π΅Π½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ ΠΊΠΈΡΡΠ΅ΠΉ, Π½Π΅ ΠΎΡΠΌΠ΅ΡΠ΅Π½ΠΎ. ΠΠ±ΡΠ΅ΠΌ ΠΎΠΏΡΡ
ΠΎΠ»Π΅Π²ΡΡ
ΡΠ·Π»ΠΎΠ² ΡΠΌΠ΅Π½ΡΡΠΈΠ»ΡΡ, ΡΡΠΎ ΠΏΠΎΠ·Π²ΠΎΠ»ΠΈΠ»ΠΎ Π±Π΅Π·ΠΎΠΏΠ°ΡΠ½ΠΎ ΠΏΡΠΎΠ²Π΅ΡΡΠΈ ΠΊΠΎΠ½ΡΡΡΠ½ΡΡ ΡΠ΅Π·Π΅ΠΊΡΠΈΡ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠΈ. ΠΡΠΈ Π½Π΅ΠΎΠΏΠ΅ΡΠ°Π±Π΅Π»ΡΠ½ΡΡ
ΡΠΎΡΠΌΠ°Ρ
ΠΠΠ Π ΠΈ Ρ
Π΅ΡΡΠ²ΠΈΠ·ΠΌΠ΅ Π½Π΅ΠΎΠ±Ρ
ΠΎΠ΄ΠΈΠΌ ΠΊΠΎΠΌΠΏΠ»Π΅ΠΊΡΠ½ΡΠΉ ΠΏΠΎΠ΄Ρ
ΠΎΠ΄: ΠΊΡΡΡ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π±ΠΎΠΌ Ρ ΠΏΠΎΡΠ»Π΅Π΄ΡΡΡΠΈΠΌ ΠΏΠ΅ΡΠ΅Ρ
ΠΎΠ΄ΠΎΠΌ Π½Π° ΡΠ΅ΡΠ°ΠΏΠΈΡ Π°Π»Π΅Π½Π΄ΡΠΎΠ½ΠΎΠ²ΠΎΠΉ ΠΊΠΈΡΠ»ΠΎΡΠΎΠΉ. ΠΠΎΡΠ»Π΅ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½Π½ΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ Π²ΡΠΏΠΎΠ»Π½Π΅Π½ΠΈΠ΅ ΠΊΠΎΠ½ΡΡΡΠ½ΠΎΠΉ ΡΠ΅Π·Π΅ΠΊΡΠΈΠΈ ΠΈΠ·Π±ΡΡΠΎΡΠ½ΠΎΠΉ ΠΊΠΎΡΡΠ½ΠΎΠΉ ΡΠΊΠ°Π½ΠΈ Ρ ΡΠ΅Π»ΡΡ ΠΌΠ΅Π΄ΠΈΠΊΠΎ-ΡΠΎΡΠΈΠ°Π»ΡΠ½ΠΎΠΉ ΡΠ΅Π°Π±ΠΈΠ»ΠΈΡΠ°ΡΠΈΠΈ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ°
Involute Helical-bevel Gearing
AbstractThis paper describes helical-bevel gearing (HBG). The helical-bevel gearing commonly includes a cylindrical and a bevel gear. The tooth profiles can be either spur or helical. The involute HBG provides an opportunity to design a broad variety of gear trains, unobtainable by the use of conventional types of gears. The article defines methods of their generation, a contact shape and points out advantages of various arrangements of gears, diagrams of an external helical-bevel gearing skew axis, hypoid gearing, intersecting axes, parallel axes. The expediency of the use of such gear in a machine drive is justified
10-Π»Π΅ΡΠ½ΠΈΠΉ ΠΎΠΏΡΡ Π»Π΅ΡΠ΅Π½ΠΈΡ ΡΠΈΠ±ΡΠΎΠ·Π½ΠΎΠΉ Π΄ΠΈΡΠΏΠ»Π°Π·ΠΈΠΈ ΡΠ΅Π»ΡΡΡΠ½ΠΎ-Π»ΠΈΡΠ΅Π²ΠΎΠΉ ΠΎΠ±Π»Π°ΡΡΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ°ΠΌΠΈ Π°Π»Π΅Π½Π΄ΡΠΎΠ½ΠΎΠ²ΠΎΠΉ ΠΊΠΈΡΠ»ΠΎΡΡ
Fibrous dysplasia is a benign rare hamartom disease of bones, characterized by a combination of fibrous tissue elements in the focis. At the present time surgical treatment is the most preferable, however, in the case of small foci or subtotal skull bone affected this approach is not rational or impossible. The accumulated foreign and our experience of conservative treatment of patients with bisphosphonate series increases the possibilities for choosing the tactics of treating patients with this congenital pathology. When using this drug, there was no development of serious adverse effects and in a number of cases, surgical treatment was avoided.Π€ΠΈΠ±ΡΠΎΠ·Π½Π°Ρ Π΄ΠΈΡΠΏΠ»Π°Π·ΠΈΡ (Π€Π) β ΡΠ΅Π΄ΠΊΠΎΠ΅ Π΄ΠΎΠ±ΡΠΎΠΊΠ°ΡΠ΅ΡΡΠ²Π΅Π½Π½ΠΎΠ΅ Π³Π°ΠΌΠ°ΡΡΠΎΠΌΠ½ΠΎΠ΅ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ΅ΠΉ, Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΡΡΠ΅Π΅ΡΡ ΡΠΎΠ΅Π΄ΠΈΠ½Π΅Π½ΠΈΠ΅ΠΌ ΡΠΈΠ±ΡΠΎΠ·Π½ΡΡ
ΠΊΠΎΡΡΠ½ΡΡ
ΡΠ»Π΅ΠΌΠ΅Π½ΡΠΎΠ² Π² ΠΎΡΠ°Π³Π΅. Π ΡΠ²ΡΠ·ΠΈ Ρ ΠΎΡΡΡΡΡΡΠ²ΠΈΠ΅ΠΌ ΠΎΠ±ΡΠ΅ΠΏΡΠΈΠ½ΡΡΠΎΠ³ΠΎ ΡΠ½ΠΈΠ²Π΅ΡΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΏΠΎΠ΄Ρ
ΠΎΠ΄Π° ΠΊ Π»Π΅ΡΠ΅Π½ΠΈΡ Π€Π Ρ
ΠΈΡΡΡΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ΅ Π»Π΅ΡΠ΅Π½ΠΈΠ΅ ΠΎΡΡΠ°Π΅ΡΡΡ ΠΏΡΠ΅Π΄ΠΏΠΎΡΡΠΈΡΠ΅Π»ΡΠ½ΡΠΌ, ΠΎΠ΄Π½Π°ΠΊΠΎ Π² ΡΠ»ΡΡΠ°Π΅ ΠΌΠ°Π»ΡΡ
ΠΎΡΠ°Π³ΠΎΠ² ΠΈΠ»ΠΈ ΡΡΠ±ΡΠΎΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΡ ΠΊΠΎΡΡΠ΅ΠΉ Ρ
ΠΈΡΡΡΠ³ΠΈΡΠ΅ΡΠΊΠΈΠΉ ΠΌΠ΅ΡΠΎΠ΄ Π½Π΅ΡΠ°ΡΠΈΠΎΠ½Π°Π»Π΅Π½ ΠΈΠ»ΠΈ Π½Π΅Π²ΠΎΠ·ΠΌΠΎΠΆΠ΅Π½. ΠΠ°ΠΊΠΎΠΏΠ»Π΅Π½Π½ΡΠΉ Π·Π°ΡΡΠ±Π΅ΠΆΠ½ΡΠΉ ΠΈ Π½Π°Ρ ΡΠΎΠ±ΡΡΠ²Π΅Π½Π½ΡΠΉ ΠΎΠΏΡΡ ΠΊΠΎΠ½ΡΠ΅ΡΠ²Π°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ°ΠΌΠΈ Π±ΠΈΡΡΠΎΡΡΠΎΠ½Π°ΡΠ½ΠΎΠ³ΠΎ ΡΡΠ΄Π° ΡΠ°ΡΡΠΈΡΡΠ΅Ρ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΡΡΠΈ Π²ΡΠ±ΠΎΡΠ° ΡΠ°ΠΊΡΠΈΠΊΠΈ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ Π΄Π°Π½Π½ΠΎΠΉ Π²ΡΠΎΠΆΠ΄Π΅Π½Π½ΠΎΠΉ ΠΏΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ. ΠΡΠΈ ΠΈΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½ΠΈΠΈ Π±ΠΈΡΡΠΎΡΡΠΎΠ½Π°ΡΠ½ΡΡ
ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠ² Π½Π΅ ΠΎΡΠΌΠ΅ΡΠ΅Π½ΠΎ ΡΠ°Π·Π²ΠΈΡΠΈΡ ΡΠ΅ΡΡΠ΅Π·Π½ΡΡ
ΠΏΠΎΠ±ΠΎΡΠ½ΡΡ
ΡΠ΅Π°ΠΊΡΠΈΠΉ, Π° Π² ΡΡΠ΄Π΅ ΡΠ»ΡΡΠ°Π΅Π² ΡΠ΄Π°Π»ΠΎΡΡ ΠΈΠ·Π±Π΅ΠΆΠ°ΡΡ ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ
Π‘ΠΠΠΠ ΠΠ ΠΠΠΠΠΠΠΠ‘Π‘ΠΠ: ΠΠΠΠΠ ΠΠΠ’ΠΠ ΠΠ’Π£Π Π« Π ΠΠΠΠΠΠ§ΠΠ‘ΠΠΠ Π‘ΠΠ£Π§ΠΠ
Introduction. Aglossia / hypoglossia is a rare condition combined with underdeveled lower jaw, lack of mandibular incisors and various degrees of limb anomalies. In domestic and foreign literature, one can find only few such observations in pediatric and adult populations. This malformation is met in the syndrome of limb and oromandibular hypogynesia or aglossia-adactylia syndrome. The incidence of aglossia / hypoglossia is very low (1 / 175,000-1 / 1,000,000 live births, ) and in most cases it is represented by hypoglosia, and not true aglossia. The isolated aglossia without limb malformations is an even more rare condition. The etiology of aglossia / hypoglosia is unknown, since all known cases are sporadic and by present moment no more than 100 observations have been published. Material and methods. In 2015, a girl (1 year 10 mon old ) and a 3- year old boy were examined in hospital and outpatiently because of complaints for the reduction of lower jaw. The children were examined by an orthodontist; additional examinations were prescribed to the girl because of revealed comorbidities. Results. The girl also had vertebrae malformation. Such a pathological combination had not been seen previously in the available literature. The second child was a boy of 3 y.o.; his parents decided to consult an orthodontist for correcting the bite. In both cases, children were well adapted, although there were some deficiencies in verbal activity and difficulties in feeding; after speech therapy and spoon-feeding trainings these problems were solved. Conclusion. Even with a significant degree of tongue underdevelopment, there are practically no functional limitations in patients with aglossia-adactyl syndrome, which is important for determining prognosis as well as for consulting patients and their parents and for making decision about the need for corrective surgery.ΠΠ²Π΅Π΄Π΅Π½ΠΈΠ΅: ΠΠ³Π»ΠΎΡΡΠΈΡ / Π³ΠΈΠΏΠΎΠ³Π»ΠΎΡΡΠΈΡ - ΡΠ΅Π΄ΠΊΠΎΠ΅ ΡΠΎΡΡΠΎΡΠ½ΠΈΠ΅, ΡΠΎΡΠ΅ΡΠ°ΡΡΠ΅Π΅ΡΡ Ρ Π½Π΅Π΄ΠΎΡΠ°Π·Π²ΠΈΡΠΈΠ΅ΠΌ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠΈ, ΠΎΡΡΡΡΡΡΠ²ΠΈΠ΅ΠΌ Π½ΠΈΠΆΠ½ΠΈΡ
ΡΠ΅Π·ΡΠΎΠ² ΠΈ ΡΠ°Π·Π»ΠΈΡΠ½ΠΎΠΉ ΡΡΠ΅ΠΏΠ΅Π½ΡΡ Π°Π½ΠΎΠΌΠ°Π»ΠΈΠΉ ΠΊΠΎΠ½Π΅ΡΠ½ΠΎΡΡΠ΅ΠΉ. Π ΠΎΡΠ΅ΡΠ΅ΡΡΠ²Π΅Π½Π½ΠΎΠΉ ΠΈ Π·Π°ΡΡΠ±Π΅ΠΆΠ½ΠΎΠΉ Π»ΠΈΡΠ΅ΡΠ°ΡΡΡΠ΅ ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Ρ Π΅Π΄ΠΈΠ½ΠΈΡΠ½ΡΠ΅ Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΡ Π΄Π΅ΡΠ΅ΠΉ ΠΈ Π²Π·ΡΠΎΡΠ»ΡΡ
Ρ Π΄Π°Π½Π½ΡΠΌ ΠΏΠΎΡΠΎΠΊΠΎΠΌ ΡΠ°Π·Π²ΠΈΡΠΈΡ, Π²Ρ
ΠΎΠ΄ΡΡΠΈΠΌ Π² ΡΠΎΡΡΠ°Π² ΡΠΈΠ½Π΄ΡΠΎΠΌΠ° Π³ΠΈΠΏΠΎΠ³Π΅Π½Π΅Π·ΠΈΠΈ ΠΊΠΎΠ½Π΅ΡΠ½ΠΎΡΡΠ΅ΠΉ ΠΈ ΠΎΡΠΎΠΌΠ°Π½Π΄ΠΈΠ±ΡΠ»ΡΡΠ½ΠΎΠΉ ΠΎΠ±Π»Π°ΡΡΠΈ (ΠΠΠΠ) ΠΈΠ»ΠΈ ΡΠΈΠ½Π΄ΡΠΎΠΌ Π°Π³Π»ΠΎΡΠΈΠΈ-Π°Π΄Π°ΠΊΡΠΈΠ»ΠΈΠΈ. ΠΠ°Π±ΠΎΠ»Π΅Π²Π°Π΅ΠΌΠΎΡΡΡ ΠΠΠΠ ΠΎΡΠ΅Π½Ρ Π½ΠΈΠ·ΠΊΠ°Ρ (1 / 175 000-1 / 1 000 000 ΠΆΠΈΠ²ΠΎΡΠΎΠΆΠ΄Π΅Π½Π½ΡΡ
) ΠΈ Π² Π±ΠΎΠ»ΡΡΠΈΠ½ΡΡΠ²Π΅ ΡΠ»ΡΡΠ°Π΅Π² ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Π° Π³ΠΈΠΏΠΎΠ³Π»ΠΎΡΠΈΠ΅ΠΉ, Π° Π½Π΅ ΠΈΡΡΠΈΠ½Π½ΠΎΠΉ Π°Π³Π»ΠΎΡΡΠΈΠ΅ΠΉ. ΠΠ·ΠΎΠ»ΠΈΡΠΎΠ²Π°Π½Π½Π°Ρ Π°Π³Π»ΠΎΡΡΠΈΡ Π±Π΅Π· ΠΏΠΎΡΠΎΠΊΠΎΠ² ΡΠ°Π·Π²ΠΈΡΠΈΡ ΠΊΠΎΠ½Π΅ΡΠ½ΠΎΡΡΠ΅ΠΉ Π΅ΡΠ΅ Π±ΠΎΠ»Π΅Π΅ ΡΠ΅Π΄ΠΊΠΎΠ΅ ΡΠΎΡΡΠΎΡΠ½ΠΈΠ΅. ΠΡΠΈΠΎΠ»ΠΎΠ³ΠΈΡ Π°Π³Π»ΠΎΡΡΠΈΠΈ / Π³ΠΈΠΏΠΎΠ³Π»ΠΎΡΠΈΠΈ Π½Π΅ΠΈΠ·Π²Π΅ΡΡΠ½Π°, ΡΠ°ΠΊ ΠΊΠ°ΠΊ Π²ΡΠ΅ ΠΈΠ·Π²Π΅ΡΡΠ½ΡΠ΅ ΡΠ»ΡΡΠ°ΠΈ Π΅Π΄ΠΈΠ½ΠΈΡΠ½Ρ ΠΈ Π½Π° Π΄Π°Π½Π½ΡΠΉ ΠΌΠΎΠΌΠ΅Π½Ρ ΠΎΠΏΡΠ±Π»ΠΈΠΊΠΎΠ²Π°Π½ΠΎ Π½Π΅ Π±ΠΎΠ»Π΅Π΅ 100 Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΠΉ. ΠΠ°ΡΠ΅ΡΠΈΠ°Π» ΠΈ ΠΌΠ΅ΡΠΎΠ΄Ρ. Π 2015 Π³. ΡΡΠ°ΡΠΈΠΎΠ½Π°ΡΠ½ΠΎ ΠΈ Π°ΠΌΠ±ΡΠ»Π°ΡΠΎΡΠ½ΠΎ Π½Π°Π±Π»ΡΠ΄Π°Π»ΠΈΡΡ ΠΈ ΠΎΠ±ΡΠ»Π΅Π΄ΠΎΠ²Π°Π»ΠΈΡΡ Π΄Π΅Π²ΠΎΡΠΊΠ° 1 Π³. 10 ΠΌΠ΅Ρ ΠΈ ΠΌΠ°Π»ΡΡΠΈΠΊ 3 Π»Π΅Ρ Ρ ΠΆΠ°Π»ΠΎΠ±Π°ΠΌΠΈ Π½Π° ΡΠΌΠ΅Π½ΡΡΠ΅Π½ΠΈΠ΅ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠΈ. ΠΠ΅ΡΠΈ ΠΎΡΠΌΠΎΡΡΠ΅Π½Ρ ΠΎΡΡΠΎΠ΄ΠΎΠ½ΡΠΎΠΌ, ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½Ρ Π΄ΠΎΠΏΠΎΠ»Π½ΠΈΡΠ΅Π»ΡΠ½ΡΠ΅ ΠΎΠ±ΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ Π² ΡΠ²ΡΠ·ΠΈ Ρ Π½Π°Π»ΠΈΡΠΈΠ΅ΠΌ ΡΠΎΠΏΡΡΡΡΠ²ΡΡΡΠ΅ΠΉ ΠΏΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠΈ Ρ Π΄Π΅Π²ΠΎΡΠΊΠΈ . Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ. Π ΠΎΠ΄Π½ΠΎΠΌ ΡΠ»ΡΡΠ°Π΅ Ρ Π΄Π΅Π²ΠΎΡΠΊΠΈ, ΠΊΡΠΎΠΌΠ΅ Π°Π½ΠΎΠΌΠ°Π»ΠΈΠΈ ΡΠ°Π·Π²ΠΈΡΠΈΡ ΡΠ·ΡΠΊΠ° ΠΈ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠΈ, Π±ΡΠ» ΠΏΠΎΡΠΎΠΊ ΡΠ°Π·Π²ΠΈΡΠΈΡ ΠΏΠΎΠ·Π²ΠΎΠ½ΠΊΠΎΠ². Π’Π°ΠΊΠΎΠ΅ ΡΠΎΡΠ΅ΡΠ°Π½ΠΈΠ΅ ΡΠ°Π½Π΅Π΅ Π½Π΅ Π²ΡΡΡΠ΅ΡΠ°Π»ΠΎΡΡ Π½Π°ΠΌ Π² Π΄ΠΎΡΡΡΠΏΠ½ΠΎΠΉ Π»ΠΈΡΠ΅ΡΠ°ΡΡΡΠ΅. ΠΡΠΎΡΡΠΌ ΡΠ΅Π±Π΅Π½ΠΊΠΎΠΌ Π±ΡΠ» ΠΌΠ°Π»ΡΡΠΈΠΊ 3 Π»Π΅Ρ, Ρ ΠΊΠΎΡΠΎΡΡΠΌ ΡΠΎΠ΄ΠΈΡΠ΅Π»ΠΈ ΠΎΠ±ΡΠ°ΡΠΈΠ»ΠΈΡΡ ΠΊ ΠΎΡΡΠΎΠ΄ΠΎΠ½ΡΡ Π΄Π»Ρ ΠΈΡΠΏΡΠ°Π²Π»Π΅Π½ΠΈΡ ΠΏΡΠΈΠΊΡΡΠ°. Π ΠΎΠ±ΠΎΠΈΡ
ΡΠ»ΡΡΠ°ΡΡ
Π΄Π΅ΡΠΈ Π±ΡΠ»ΠΈ Ρ
ΠΎΡΠΎΡΠΎ Π°Π΄Π°ΠΏΡΠΈΡΠΎΠ²Π°Π½Ρ, Ρ
ΠΎΡΡ ΠΎΡΠΌΠ΅ΡΠ°Π»ΡΡ Π½Π΅ΠΊΠΎΡΠΎΡΡΠΉ Π΄Π΅ΡΠΈΡΠΈΡ Π²Π΅ΡΠ±Π°Π»ΡΠ½ΠΎΠΉ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ ΠΈ ΡΡΡΠ΄Π½ΠΎΡΡΠΈ ΠΏΡΠΈ ΠΊΠΎΡΠΌΠ»Π΅Π½ΠΈΠΈ, ΠΊΠΎΡΠΎΡΡΠ΅ ΠΏΠΎΡΠ»Π΅ Π»ΠΎΠ³ΠΎΠΏΠ΅Π΄ΠΈΡΠ΅ΡΠΊΠΎΠΉ ΠΊΠΎΡΡΠ΅ΠΊΡΠΈΠΈ ΠΈ ΠΎΠ±ΡΡΠ΅Π½ΠΈΡ ΠΊΠΎΡΠΌΠ»Π΅Π½ΠΈΡ Ρ Π»ΠΎΠΆΠΊΠΈ Π±ΡΠ»ΠΈ ΡΠ΅ΡΠ΅Π½Ρ. ΠΠ°ΠΊΠ»ΡΡΠ΅Π½ΠΈΠ΅. ΠΠ°ΠΆΠ΅ ΠΏΡΠΈ Π·Π½Π°ΡΠΈΡΠ΅Π»ΡΠ½ΠΎΠΉ ΡΡΠ΅ΠΏΠ΅Π½ΠΈ Π½Π΅Π΄ΠΎΡΠ°Π·Π²ΠΈΡΠΈΡ ΡΠ·ΡΠΊΠ°, ΡΡΠ½ΠΊΡΠΈΠΎΠ½Π°Π»ΡΠ½ΡΠ΅ ΠΎΠ³ΡΠ°Π½ΠΈΡΠ΅Π½ΠΈΡ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠΈΠ½Π΄ΡΠΎΠΌΠΎΠΌ Π°Π³Π»ΠΎΡΡΠΈΠΈ-Π°Π΄Π°ΠΊΡΠΈΠ»ΠΈΠΈ ΠΏΡΠ°ΠΊΡΠΈΡΠ΅ΡΠΊΠΈ ΠΎΡΡΡΡΡΡΠ²ΡΡΡ, ΡΡΠΎ Π²Π°ΠΆΠ½ΠΎ Π΄Π»Ρ ΠΎΠΏΡΠ΅Π΄Π΅Π»Π΅Π½ΠΈΡ ΠΏΡΠΎΠ³Π½ΠΎΠ·Π°, Π° ΡΠ°ΠΊΠΆΠ΅ ΠΏΡΠΈ ΠΊΠΎΠ½ΡΡΠ»ΡΡΠ°ΡΠΈΠΈ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ°ΠΌΠΈ ΠΈ ΠΈΡ
ΡΠΎΠ΄ΠΈΡΠ΅Π»ΡΠΌΠΈ ΠΈ ΠΏΡΠΈΠ½ΡΡΠΈΡ ΡΠ΅ΡΠ΅Π½ΠΈΠΉ ΠΎ Π½Π΅ΠΎΠ±Ρ
ΠΎΠ΄ΠΈΠΌΠΎΡΡΠΈ ΠΊΠΎΡΡΠ΅ΠΊΡΠΈΡΡΡΡΠΈΡ
ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠΉ
Π’Π°ΡΠ³Π΅ΡΠ½Π°Ρ ΡΠ΅ΡΠ°ΠΏΠΈΡ Ρ Π΅ΡΡΠ²ΠΈΠ·ΠΌΠ° Ρ ΡΠ΅Π±Π΅Π½ΠΊΠ° 9 Π»Π΅Ρ
Reparative giant cell granulomas (RGCG) is a rare benign lesion of the skull bones in children, which is 1.7% of all recorded cases of RGCG. Despite the benign nature of the disease, RGCG often recurs after surgical treatment and can metastasize. One of the rare forms of RGCG is cherubism, characterized by total defeat of the upper and lower jaw, in connection with which it is impossible to carry out radical surgical treatment, especially in children in the period before puberty. In the department of maxillofacial surgery of the Russian Childrensβ Clinical Hospital under ambulatory supervision for 3 years was a child diagnosed with cherubism. During this time, progressive growth of the upper and lower jaw persisted, leading to exorbitalism. After the histological verification of the diagnosis and the approval of the ethical committee, a course of therapy with the drug denosumab was conducted. Histological examination of the tumor denosumab treatment showed a significant response which resulted in disappearance of giant cells. CT before and after the therapy showed an increase in bone density from 65 to 385 HU. The size of tumor nodes reduced, allowing to made contour resection of the mandible. The structure and volume of the upper jaw reached the average value and did not require surgical correction. After the course of the denosumab, there was no growth retardation according to the control X-ray examination of the wrist. inoperable forms of RGCG and cherubism, a complex approach is needed, including a course of denosumab therapy followed by transfer to therapy with alendronic acid. After the therapy, contour resection of excess bone tissue is possible with the purpose of medical and social rehabilitation of such patients.ΠΠΈΠ³Π°Π½ΡΠΎΠΊΠ»Π΅ΡΠΎΡΠ½Π°Ρ ΡΠ΅ΠΏΠ°ΡΠ°ΡΠΈΠ²Π½Π°Ρ Π³ΡΠ°Π½ΡΠ»Π΅ΠΌΠ° (ΠΠΠ Π) β ΡΠ΅Π΄ΠΊΠΎΠ΅ Π΄ΠΎΠ±ΡΠΎΠΊΠ°ΡΠ΅ΡΡΠ²Π΅Π½Π½ΠΎΠ΅ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ΅ΠΉ ΡΠ΅ΡΠ΅ΠΏΠ° Ρ Π΄Π΅ΡΠ΅ΠΉ, ΠΊΠΎΡΠΎΡΠΎΠ΅ ΡΠΎΡΡΠ°Π²Π»ΡΠ΅Ρ 1,7% Π²ΡΠ΅Ρ
ΡΠ΅Π³ΠΈΡΡΡΠΈΡΡΠ΅ΠΌΡΡ
ΡΠ»ΡΡΠ°Π΅Π² ΠΠΠ Π. ΠΠ΅ΡΠΌΠΎΡΡΡ Π½Π° Π΄ΠΎΠ±ΡΠΎΠΊΠ°ΡΠ΅ΡΡΠ²Π΅Π½Π½ΡΠΉ Ρ
Π°ΡΠ°ΠΊΡΠ΅Ρ, ΠΠΠ Π ΡΠ°ΡΡΠΎ ΡΠ΅ΡΠΈΠ΄ΠΈΠ²ΠΈΡΡΠ΅Ρ ΠΏΠΎΡΠ»Π΅ ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΈ ΠΌΠΎΠΆΠ΅Ρ ΠΌΠ΅ΡΠ°ΡΡΠ°Π·ΠΈΡΠΎΠ²Π°ΡΡ. ΠΠ΄Π½Π° ΠΈΠ· ΡΠ΅Π΄ΠΊΠΈΡ
ΡΠΎΡΠΌ ΠΠΠ Π β Ρ
Π΅ΡΡΠ²ΠΈΠ·ΠΌ, Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠ½ΡΠΉ ΡΠΎΡΠ°Π»ΡΠ½ΡΠΌ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΠ΅ΠΌ Π²Π΅ΡΡ
Π½Π΅ΠΉ ΠΈ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠ΅ΠΉ, Π² ΡΠ²ΡΠ·ΠΈ Ρ ΡΠ΅ΠΌ Π½Π΅Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½ΠΈΠ΅ ΡΠ°Π΄ΠΈΠΊΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ, ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎ Ρ Π΄Π΅ΡΠ΅ΠΉ Π² ΠΏΠ΅ΡΠΈΠΎΠ΄ Π΄ΠΎ ΠΏΠΎΠ»ΠΎΠ²ΠΎΠ³ΠΎ ΡΠΎΠ·ΡΠ΅Π²Π°Π½ΠΈΡ. Π ΠΎΡΠ΄Π΅Π»Π΅Π½ΠΈΠΈ ΡΠ΅Π»ΡΡΡΠ½ΠΎ-Π»ΠΈΡΠ΅Π²ΠΎΠΉ Ρ
ΠΈΡΡΡΠ³ΠΈΠΈ Π ΠΎΡΡΠΈΠΉΡΠΊΠΎΠΉ Π΄Π΅ΡΡΠΊΠΎΠΉ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΉ Π±ΠΎΠ»ΡΠ½ΠΈΡΡ ΠΠΈΠ½Π·Π΄ΡΠ°Π²Π° Π ΠΎΡΡΠΈΠΈ ΠΏΠΎΠ΄ Π°ΠΌΠ±ΡΠ»Π°ΡΠΎΡΠ½ΡΠΌ Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΠ΅ΠΌ Π² ΡΠ΅ΡΠ΅Π½ΠΈΠ΅ 3 Π»Π΅Ρ Π½Π°Ρ
ΠΎΠ΄ΠΈΠ»ΡΡ ΡΠ΅Π±Π΅Π½ΠΎΠΊ Ρ Π΄ΠΈΠ°Π³Π½ΠΎΠ·ΠΎΠΌ Β«Ρ
Π΅ΡΡΠ²ΠΈΠ·ΠΌΒ»: Π½Π°Π±Π»ΡΠ΄Π°Π»ΡΡ ΠΏΡΠΎΠ³ΡΠ΅ΡΡΠΈΠ²Π½ΡΠΉ ΡΠΎΡΡ Π²Π΅ΡΡ
Π½Π΅ΠΉ ΠΈ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠ΅ΠΉ, ΠΏΡΠΈΠ²ΠΎΠ΄ΡΡΠΈΠΉ ΠΊ ΡΠΊΠ·ΠΎΠΎΡΠ±ΠΈΡΠΈΠ·ΠΌΡ. ΠΠΎΡΠ»Π΅ Π³ΠΈΡΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠΉ Π²Π΅ΡΠΈΡΠΈΠΊΠ°ΡΠΈΠΈ Π΄ΠΈΠ°Π³Π½ΠΎΠ·Π° ΠΈ ΠΏΠΎΠ»ΡΡΠ΅Π½ΠΈΡ ΠΎΠ΄ΠΎΠ±ΡΠ΅Π½ΠΈΡ ΡΡΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΠΊΠΎΠΌΠΈΡΠ΅ΡΠ° ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½ ΠΊΡΡΡ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π±. ΠΠΈΡΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ΅ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΎΠΏΡΡ
ΠΎΠ»ΠΈ ΠΏΠΎΡΠ»Π΅ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π± ΠΏΡΠΎΠ΄Π΅ΠΌΠΎΠ½ΡΡΡΠΈΡΠΎΠ²Π°Π»ΠΎ Π²ΡΡΠ°ΠΆΠ΅Π½Π½ΡΠΉ ΠΏΠ°ΡΠΎΠΌΠΎΡΡΠΎΠ·, ΠΎ ΡΠ΅ΠΌ ΡΠ²ΠΈΠ΄Π΅ΡΠ΅Π»ΡΡΡΠ²ΠΎΠ²Π°Π»ΠΈ ΠΌΠ°Π»ΠΎΡΠΈΡΠ»Π΅Π½Π½ΠΎΡΡΡ ΠΈΠ»ΠΈ ΠΈΡΡΠ΅Π·Π½ΠΎΠ²Π΅Π½ΠΈΠ΅ Π³ΠΈΠ³Π°Π½ΡΡΠΊΠΈΡ
ΠΊΠ»Π΅ΡΠΎΠΊ, ΠΎΠ±ΡΠ°Π·ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ½ΠΎΠΉ ΠΈ Π²ΠΎΠ»ΠΎΠΊΠ½ΠΈΡΡΠΎΠΉ ΡΠΊΠ°Π½Π΅ΠΉ. ΠΠΎ Π΄Π°Π½Π½ΡΠΌ ΠΊΠΎΠΌΠΏΡΡΡΠ΅ΡΠ½ΠΎΠΉ ΡΠΎΠΌΠΎΠ³ΡΠ°ΡΠΈΠΈ Π΄ΠΎ ΠΈ ΠΏΠΎΡΠ»Π΅ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ, ΠΎΡΠΌΠ΅ΡΠ΅Π½ΠΎ ΠΏΠΎΠ²ΡΡΠ΅Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ½ΠΎΠΉ ΠΏΠ»ΠΎΡΠ½ΠΎΡΡΠΈ Ρ 65 Π΄ΠΎ 385 HU. ΠΠΎΡΠ»Π΅ ΠΊΡΡΡΠ° ΡΠ΅ΡΠ°ΠΏΠΈΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠΌ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π± Π·Π°ΠΌΠ΅Π΄Π»Π΅Π½ΠΈΡ ΡΠΎΡΡΠ°, ΠΏΠΎ Π΄Π°Π½Π½ΡΠΌ ΠΊΠΎΠ½ΡΡΠΎΠ»ΡΠ½ΠΎΠ³ΠΎ ΡΠ΅Π½ΡΠ³Π΅Π½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ ΠΊΠΈΡΡΠ΅ΠΉ, Π½Π΅ ΠΎΡΠΌΠ΅ΡΠ΅Π½ΠΎ. ΠΠ±ΡΠ΅ΠΌ ΠΎΠΏΡΡ
ΠΎΠ»Π΅Π²ΡΡ
ΡΠ·Π»ΠΎΠ² ΡΠΌΠ΅Π½ΡΡΠΈΠ»ΡΡ, ΡΡΠΎ ΠΏΠΎΠ·Π²ΠΎΠ»ΠΈΠ»ΠΎ Π±Π΅Π·ΠΎΠΏΠ°ΡΠ½ΠΎ ΠΏΡΠΎΠ²Π΅ΡΡΠΈ ΠΊΠΎΠ½ΡΡΡΠ½ΡΡ ΡΠ΅Π·Π΅ΠΊΡΠΈΡ Π½ΠΈΠΆΠ½Π΅ΠΉ ΡΠ΅Π»ΡΡΡΠΈ. ΠΡΠΈ Π½Π΅ΠΎΠΏΠ΅ΡΠ°Π±Π΅Π»ΡΠ½ΡΡ
ΡΠΎΡΠΌΠ°Ρ
ΠΠΠ Π ΠΈ Ρ
Π΅ΡΡΠ²ΠΈΠ·ΠΌΠ΅ Π½Π΅ΠΎΠ±Ρ
ΠΎΠ΄ΠΈΠΌ ΠΊΠΎΠΌΠΏΠ»Π΅ΠΊΡΠ½ΡΠΉ ΠΏΠΎΠ΄Ρ
ΠΎΠ΄: ΠΊΡΡΡ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ Π΄Π΅Π½ΠΎΠ·ΡΠΌΠ°Π±ΠΎΠΌ Ρ ΠΏΠΎΡΠ»Π΅Π΄ΡΡΡΠΈΠΌ ΠΏΠ΅ΡΠ΅Ρ
ΠΎΠ΄ΠΎΠΌ Π½Π° ΡΠ΅ΡΠ°ΠΏΠΈΡ Π°Π»Π΅Π½Π΄ΡΠΎΠ½ΠΎΠ²ΠΎΠΉ ΠΊΠΈΡΠ»ΠΎΡΠΎΠΉ. ΠΠΎΡΠ»Π΅ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½Π½ΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ Π²ΡΠΏΠΎΠ»Π½Π΅Π½ΠΈΠ΅ ΠΊΠΎΠ½ΡΡΡΠ½ΠΎΠΉ ΡΠ΅Π·Π΅ΠΊΡΠΈΠΈ ΠΈΠ·Π±ΡΡΠΎΡΠ½ΠΎΠΉ ΠΊΠΎΡΡΠ½ΠΎΠΉ ΡΠΊΠ°Π½ΠΈ Ρ ΡΠ΅Π»ΡΡ ΠΌΠ΅Π΄ΠΈΠΊΠΎ-ΡΠΎΡΠΈΠ°Π»ΡΠ½ΠΎΠΉ ΡΠ΅Π°Π±ΠΈΠ»ΠΈΡΠ°ΡΠΈΠΈ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠ°
ΠΡΠ΅Π΄ΡΡΠ½ΡΠ΅ ΡΠ²ΠΈΡΠΈ: ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠ΅ Π²Π°ΡΠΈΠ°Π½ΡΡ ΠΈ ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠ²Π½ΠΎΠ΅ Π»Π΅ΡΠ΅Π½ΠΈΠ΅
Introduction. Preauricular sinuses and cysts are a frequent congenital head and neck organs anomaly in children, which appears in the form of a small hole near the anterior limb of the ascending helix. Despite a seemingly easy surgical treatment for these cysts, the relapse rate stays high. The purpose of this article is to analyze the value of diagnostic methods in planning surgical treatment of preauricular sinuses. Material and methods. A retrospective study was conducted based on data obtained from the Craniomaxillofacial Surgery Department of the Russian Children's Clinical Hospital, Moscow, from 2012 to 2019. Medical records were checked for the facts of postoperative complications and relapse rates. The fistulous canal was visualized using preoperative fistulography with computed tomography, as well as intraoperative instillation of the dye and installation of the catheter. The skin near the sinus was excised elliptically, and the sinus was removed throughout the dyed area. Results. 17 patients aged from 9 months to 14.5 years were included in the study. A unilateral preauricular sinus tract was right-sided in 7 patients and left-sided in 10 patients). None of the patients had problems with the wound in the postoperative period. However, in 1 case there was relapse after 1 year. Conclusion. Using fistulography, a combination of dye instillation, insertion of a catheter and modified dissection, it is possible to achieve a relapse-free series of excision of the preauricular fistula among a heterogeneous group of patients.ΠΠ²Π΅Π΄Π΅Π½ΠΈΠ΅. ΠΡΠ΅Π°ΡΡΠΈΠΊΡΠ»ΡΡΠ½ΡΠ΅ ΡΠ²ΠΈΡΠΈ ΠΈ ΠΊΠΈΡΡΡ - ΠΎΠ΄Π½Π° ΠΈΠ· ΡΠ°ΡΡΡΡ
Π²ΡΠΎΠΆΠ΄Π΅Π½Π½ΡΡ
Π°Π½ΠΎΠΌΠ°Π»ΠΈΠΉ Π³ΠΎΠ»ΠΎΠ²Ρ ΠΈ ΡΠ΅ΠΈ Ρ Π΄Π΅ΡΠ΅ΠΉ, ΠΏΡΠΎΡΠ²Π»ΡΡΡΠ°ΡΡΡ Π² Π²ΠΈΠ΄Π΅ Π½Π΅Π±ΠΎΠ»ΡΡΠΎΠ³ΠΎ ΠΎΡΠ²Π΅ΡΡΡΠΈΡ Π²Π±Π»ΠΈΠ·ΠΈ ΠΏΠ΅ΡΠ΅Π΄Π½Π΅ΠΉ Π½ΠΎΠΆΠΊΠΈ Π·Π°Π²ΠΈΡΠΊΠ°. ΠΠ΅ΡΠΌΠΎΡΡΡ Π½Π° ΠΊΠ°ΠΆΡΡΠ΅ΡΡΡ ΠΏΡΠΎΡΡΠΎΡΡ Ρ
ΠΈΡΡΡΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΡΡΠΈΡ
ΠΊΠΈΡΡ, ΡΠ°ΡΡΠΎΡΠ° ΡΠ°Π·Π²ΠΈΡΠΈΡ ΡΠ΅ΡΠΈΠ΄ΠΈΠ²ΠΎΠ² ΠΎΡΡΠ°Π΅ΡΡΡ Π²ΡΡΠΎΠΊΠΎΠΉ. ΠΠ°ΡΠ΅ΡΠΈΠ°Π» ΠΈ ΠΌΠ΅ΡΠΎΠ΄Ρ. ΠΡΠΎΠ²Π΅Π΄Π΅Π½ΠΎ ΡΠ΅ΡΡΠΎΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠ΅ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ Π½Π° Π±Π°Π·Π΅ ΠΎΡΠ΄Π΅Π»Π΅Π½ΠΈΡ ΡΠ΅Π»ΡΡΡΠ½ΠΎ-Π»ΠΈΡΠ΅Π²ΠΎΠΉ Ρ
ΠΈΡΡΡΠ³ΠΈΠΈ Π ΠΎΡΡΠΈΠΉΡΠΊΠΎΠΉ Π΄Π΅ΡΡΠΊΠΎΠΉ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΉ Π±ΠΎΠ»ΡΠ½ΠΈΡΡ (Π ΠΠΠ) ΠΠΎΡΠΊΠ²Ρ Ρ 2012 ΠΏΠΎ 2019 Π³. ΠΠ΅Π΄ΠΈΡΠΈΠ½ΡΠΊΠΈΠ΅ Π·Π°ΠΏΠΈΡΠΈ Π±ΡΠ»ΠΈ ΠΏΡΠΎΠ²Π΅ΡΠ΅Π½Ρ Π½Π° ΠΏΡΠ΅Π΄ΠΌΠ΅Ρ ΠΏΠΎΡΠ»Π΅ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠΎΠ½Π½ΡΡ
ΠΎΡΠ»ΠΎΠΆΠ½Π΅Π½ΠΈΠΉ ΠΈ ΡΠ°ΡΡΠΎΡΡ ΡΠ΅ΡΠΈΠ΄ΠΈΠ²ΠΎΠ². Π‘Π²ΠΈΡΠ΅Π²ΠΎΠΉ ΠΊΠ°Π½Π°Π» Π²ΠΈΠ·ΡΠ°Π»ΠΈΠ·ΠΈΡΠΎΠ²Π°Π»ΠΈ Ρ ΠΏΠΎΠΌΠΎΡΡΡ ΠΏΡΠ΅Π΄ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠΎΠ½Π½ΠΎΠΉ ΡΠΈΡΡΡΠ»ΠΎΠ³ΡΠ°ΡΠΈΠΈ Ρ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½ΠΈΠ΅ΠΌ ΠΊΠΎΠΌΠΏΡΡΡΠ΅ΡΠ½ΠΎΠΉ ΡΠΎΠΌΠΎΠ³ΡΠ°ΡΠΈΠΈ, Π° ΡΠ°ΠΊΠΆΠ΅ ΠΈΠ½ΡΡΠ°ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠΎΠ½Π½ΠΎ Π·Π°ΠΊΠ°ΠΏΡΠ²Π°Π»ΠΈ ΠΊΡΠ°ΡΠΈΡΠ΅Π»Ρ ΠΈ ΡΡΡΠ°Π½Π°Π²Π»ΠΈΠ²Π°Π»ΠΈ Π·ΠΎΠ½Π΄. ΠΠΎΠΆΠ° ΡΡΠ΄ΠΎΠΌ Ρ ΠΎΡΠ²Π΅ΡΡΡΠΈΠ΅ΠΌ ΠΏΠ°Π·ΡΡ
ΠΈ ΡΠ°ΡΡΠ΅ΠΊΠ°Π»Π°ΡΡ ΡΠ»Π»ΠΈΠΏΡΠΈΡΠ΅ΡΠΊΠΈ, Π° ΡΠ°ΠΌ ΡΠ²ΠΈΡΠ΅Π²ΠΎΠΉ Ρ
ΠΎΠ΄ ΠΈΡΡΠ΅ΠΊΠ°Π»ΡΡ Π½Π° Π²ΡΠ΅ΠΌ ΠΏΡΠΎΡΡΠΆΠ΅Π½ΠΈΠΈ ΠΏΡΠΎΠΊΡΠ°ΡΠΈΠ²Π°Π½ΠΈΡ. Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ. Π ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ Π±ΡΠ»ΠΈ ΠΎΡΠΎΠ±ΡΠ°Π½Ρ 17 ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π² Π²ΠΎΠ·ΡΠ°ΡΡΠ΅ ΠΎΡ 9 ΠΌΠ΅ΡΡΡΠ΅Π² Π΄ΠΎ 14,5 Π³ΠΎΠ΄Π°. ΠΠ΄Π½ΠΎΡΡΠΎΡΠΎΠ½Π½ΠΈΠΉ ΠΏΡΠ΅Π°ΡΡΠΈΠΊΡΠ»ΡΡΠ½ΡΠΉ ΡΠΈΠ½ΡΡΠΎΠ²ΡΠΉ ΡΡΠ°ΠΊΡ ΠΏΡΠΈΡΡΡΡΡΠ²ΠΎΠ²Π°Π» Ρ 7 ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΡΠΏΡΠ°Π²Π° ΠΈ Ρ 10 ΡΠ»Π΅Π²Π°. ΠΠΈ Ρ ΠΎΠ΄Π½ΠΎΠ³ΠΎ ΠΈΠ· ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π½Π΅ Π±ΡΠ»ΠΎ ΠΏΡΠΎΠ±Π»Π΅ΠΌ Ρ ΡΠ°Π½ΠΎΠΉ Π² ΠΏΠΎΡΠ»Π΅ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠΎΠ½Π½ΠΎΠΌ ΠΏΠ΅ΡΠΈΠΎΠ΄Π΅, ΠΎΠ΄Π½Π°ΠΊΠΎ Π² 1 ΡΠ»ΡΡΠ°Π΅ ΡΠ°Π·Π²ΠΈΠ»ΡΡ ΡΠ΅ΡΠΈΠ΄ΠΈΠ² ΡΠ΅ΡΠ΅Π· 1 Π³ΠΎΠ΄. ΠΠ°ΠΊΠ»ΡΡΠ΅Π½ΠΈΠ΅. ΠΡΠΏΠΎΠ»ΡΠ·ΡΡ ΡΠΈΡΡΡΠ»ΠΎΠ³ΡΠ°ΡΠΈΡ, ΠΊΠΎΠΌΠ±ΠΈΠ½Π°ΡΠΈΡ ΠΈΠ½ΡΡΠΈΠ»Π»ΡΡΠΈΠΈ ΠΊΡΠ°ΡΠΈΡΠ΅Π»Ρ, Π²Π²Π΅Π΄Π΅Π½ΠΈΡ Π·ΠΎΠ½Π΄Π° ΠΈ ΠΌΠΎΠ΄ΠΈΡΠΈΡΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠΉ Π΄ΠΈΡΡΠ΅ΠΊΡΠΈΠΈ, Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ Π΄ΠΎΠ±ΠΈΡΡΡΡ Π±Π΅Π·ΡΠ΅ΡΠΈΠ΄ΠΈΠ²Π½ΠΎΠΉ ΡΠ΅ΡΠΈΠΈ ΠΈΡΡΠ΅ΡΠ΅Π½ΠΈΡ ΠΏΡΠ΅Π°ΡΡΠΈΠΊΡΠ»ΡΡΠ½ΠΎΠ³ΠΎ ΡΠ²ΠΈΡΠ° ΡΡΠ΅Π΄ΠΈ ΡΠ°Π·Π½ΠΎΡΠΎΠ΄Π½ΠΎΠΉ Π³ΡΡΠΏΠΏΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ²
Unique Experience of Cherubism Targeted Therapy
The family form of giant cell reparative granuloma or cherubism is a rare benign lesion of the jaws which causes face deformation reminiscent of the cherubs portrayed in Renaissance art. Radical surgery, especially in children before puberty, is impossible or irrational, because it leads to disablement. For four years, a child with cherubism was undergoing an outpatient supervision in the Department of Maxillofacial Surgery in Russian Children Clinical Hospital. During the observation period it was noted that tumor masses were slowly progressing leading to exoorbitism. The child had a diagnosis histological verification; a treatment using human monoclonal antibodies to RANKL was developed, adapted and approved by the ethical committee. The clinical effect was observed from the third month of therapy - jaws corners sharpened, the volume reduced. The control biopsy material taken at the end of the 6-month course did not reveal any giant cells which meant complete pathomorphism. Computed tomography showed that at the end of the therapy bone density increased by 6-7 times in the affected zones and was additionally growing over the next 6 months of observation. Such dynamics allowed making effective contouring surgery of excessive bone tissues. Inoperable and early stages of cherubism require a combined treatment which includes a course of monoclonal antibodies followed by alendronic acid therapy that increases osteoblasts survival. After the treatment, if it is necessary to improve the appearance, contouring surgery of excessive bone tissue is performed
10-Π»Π΅ΡΠ½ΠΈΠΉ ΠΎΠΏΡΡ Π»Π΅ΡΠ΅Π½ΠΈΡ ΡΠΈΠ±ΡΠΎΠ·Π½ΠΎΠΉ Π΄ΠΈΡΠΏΠ»Π°Π·ΠΈΠΈ ΡΠ΅Π»ΡΡΡΠ½ΠΎ-Π»ΠΈΡΠ΅Π²ΠΎΠΉ ΠΎΠ±Π»Π°ΡΡΠΈ ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ°ΠΌΠΈ Π°Π»Π΅Π½Π΄ΡΠΎΠ½ΠΎΠ²ΠΎΠΉ ΠΊΠΈΡΠ»ΠΎΡΡ
Fibrous dysplasia is a benign rare hamartom disease of bones, characterized by a combination of fibrous tissue elements in the focis. At the present time surgical treatment is the most preferable, however, in the case of small foci or subtotal skull bone affected this approach is not rational or impossible. The accumulated foreign and our experience of conservative treatment of patients with bisphosphonate series increases the possibilities for choosing the tactics of treating patients with this congenital pathology. When using this drug, there was no development of serious adverse effects and in a number of cases, surgical treatment was avoided.Π€ΠΈΠ±ΡΠΎΠ·Π½Π°Ρ Π΄ΠΈΡΠΏΠ»Π°Π·ΠΈΡ (Π€Π) β ΡΠ΅Π΄ΠΊΠΎΠ΅ Π΄ΠΎΠ±ΡΠΎΠΊΠ°ΡΠ΅ΡΡΠ²Π΅Π½Π½ΠΎΠ΅ Π³Π°ΠΌΠ°ΡΡΠΎΠΌΠ½ΠΎΠ΅ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠ΅ ΠΊΠΎΡΡΠ΅ΠΉ, Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΡΡΠ΅Π΅ΡΡ ΡΠΎΠ΅Π΄ΠΈΠ½Π΅Π½ΠΈΠ΅ΠΌ ΡΠΈΠ±ΡΠΎΠ·Π½ΡΡ
ΠΊΠΎΡΡΠ½ΡΡ
ΡΠ»Π΅ΠΌΠ΅Π½ΡΠΎΠ² Π² ΠΎΡΠ°Π³Π΅. Π ΡΠ²ΡΠ·ΠΈ Ρ ΠΎΡΡΡΡΡΡΠ²ΠΈΠ΅ΠΌ ΠΎΠ±ΡΠ΅ΠΏΡΠΈΠ½ΡΡΠΎΠ³ΠΎ ΡΠ½ΠΈΠ²Π΅ΡΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΏΠΎΠ΄Ρ
ΠΎΠ΄Π° ΠΊ Π»Π΅ΡΠ΅Π½ΠΈΡ Π€Π Ρ
ΠΈΡΡΡΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ΅ Π»Π΅ΡΠ΅Π½ΠΈΠ΅ ΠΎΡΡΠ°Π΅ΡΡΡ ΠΏΡΠ΅Π΄ΠΏΠΎΡΡΠΈΡΠ΅Π»ΡΠ½ΡΠΌ, ΠΎΠ΄Π½Π°ΠΊΠΎ Π² ΡΠ»ΡΡΠ°Π΅ ΠΌΠ°Π»ΡΡ
ΠΎΡΠ°Π³ΠΎΠ² ΠΈΠ»ΠΈ ΡΡΠ±ΡΠΎΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΡ ΠΊΠΎΡΡΠ΅ΠΉ Ρ
ΠΈΡΡΡΠ³ΠΈΡΠ΅ΡΠΊΠΈΠΉ ΠΌΠ΅ΡΠΎΠ΄ Π½Π΅ΡΠ°ΡΠΈΠΎΠ½Π°Π»Π΅Π½ ΠΈΠ»ΠΈ Π½Π΅Π²ΠΎΠ·ΠΌΠΎΠΆΠ΅Π½. ΠΠ°ΠΊΠΎΠΏΠ»Π΅Π½Π½ΡΠΉ Π·Π°ΡΡΠ±Π΅ΠΆΠ½ΡΠΉ ΠΈ Π½Π°Ρ ΡΠΎΠ±ΡΡΠ²Π΅Π½Π½ΡΠΉ ΠΎΠΏΡΡ ΠΊΠΎΠ½ΡΠ΅ΡΠ²Π°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠ°ΠΌΠΈ Π±ΠΈΡΡΠΎΡΡΠΎΠ½Π°ΡΠ½ΠΎΠ³ΠΎ ΡΡΠ΄Π° ΡΠ°ΡΡΠΈΡΡΠ΅Ρ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΡΡΠΈ Π²ΡΠ±ΠΎΡΠ° ΡΠ°ΠΊΡΠΈΠΊΠΈ Π»Π΅ΡΠ΅Π½ΠΈΡ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ Π΄Π°Π½Π½ΠΎΠΉ Π²ΡΠΎΠΆΠ΄Π΅Π½Π½ΠΎΠΉ ΠΏΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠ΅ΠΉ. ΠΡΠΈ ΠΈΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½ΠΈΠΈ Π±ΠΈΡΡΠΎΡΡΠΎΠ½Π°ΡΠ½ΡΡ
ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠ² Π½Π΅ ΠΎΡΠΌΠ΅ΡΠ΅Π½ΠΎ ΡΠ°Π·Π²ΠΈΡΠΈΡ ΡΠ΅ΡΡΠ΅Π·Π½ΡΡ
ΠΏΠΎΠ±ΠΎΡΠ½ΡΡ
ΡΠ΅Π°ΠΊΡΠΈΠΉ, Π° Π² ΡΡΠ΄Π΅ ΡΠ»ΡΡΠ°Π΅Π² ΡΠ΄Π°Π»ΠΎΡΡ ΠΈΠ·Π±Π΅ΠΆΠ°ΡΡ ΠΎΠΏΠ΅ΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ
Craniomaxillofacial anomalies in gorlinβgoltz syndrome: A description of clinical cases
GorlinβGoltz syndrome is an autosomal dominant rare phacomatosis characterized by multiple odontogenic keratocysts, rib splitting, multiple basal cell carcinomas and other anomalies. The most frequent early manifestation of this disease is multiple keratocysts, therefore the task of it often falls with dentists, maxillofacial and pediatric surgeons. Materials and methods: authors performed a retrospective analysis of treated patients to assess a correlation between the presence of large odontogenic cysts and GorlinβGoltz syndrome. All children underwent computed tomography at the preoperative stage, followed by surgical treatment that included cystectomy. Results: multiple or large odontogenic cysts in young patients require a complete examination with computed tomography of the entire skull to detect a calcification falx cerebri, which was found in 4 out of 7 patients with Gorlin-Goltz syndrome. In all the cases, the lower jaw was affected by keratocysts prior to foci appearing on the posterior parts of the upper jaw. The surgical treatment must include cysts resection with the embedded teeth and placement of the xeroform turunda into the tumor bed to reduce the risk of relapse. Conclusion: an early diagnosis of Gorlin-Goltz syndrome plays a crucial role in avoiding severe complications such as maxillofacial deformities associated with jaw cysts. Β© 2020, Pediatria Ltd.. All rights reserved