5 research outputs found
ΠΠΎΠ·ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠ°Ρ ΡΡΡΡΠΊΡΡΡΠ° ΠΈ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΡ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ Π»Π΅Π³ΠΊΠΈΡ Ρ Π΄Π΅ΡΠ΅ΠΉ ΠΏΠ΅ΡΠ²ΡΡ 2 Π»Π΅Ρ ΠΆΠΈΠ·Π½ΠΈ: ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΡ ΠΌΠ½ΠΎΠ³ΠΎΡΠ΅Π½ΡΡΠΎΠ²ΠΎΠ³ΠΎ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ
The article presents data about study included 76 children of the first two years of life with interstitial lung diseases (ILD). According to symptoms of chILD-syndrome, all patients had respiratory signs and interstitial changes on X-ray. 93.4% of patients had respiratory symptoms, hypoxemia was revealed in 68.4% of patients by pulsoximetry. The presence of those symptoms allowed to establish chILD-syndrome in all patients, and, on the basis of clinical and laboratory-instrumental studies, nosological form was refined. Children of our study have forms of IlD such as congenital alveolar-capillary dysplasia (1.3%), pulmonary hypoplasia (17%), Wilson-Mikity syndrome (21.1%), subpleural cysts in patients with Down syndrome (6.6%), neuroendocrine cell hyperplasia of infancy (23.7%), congenital deficiency of surfactant protein B (1.3%), brain-lung-thyroid syndrome (2.6%), bronchiolitis obliterans with organizing pneumonia (10.5%), disorders related to systemic disease processes (Langerhans cell histiocytosis - 14.6%, Niemann-Pick disease - 1.3%). The features of clinical picture, data of laboratory-instrumental methods of investigation, severity of the disease's course, prognosis, predictors of the death outcome in patients with these rare diseases, have been analyzed in all patients including preterm infants.Π ΡΡΠ°ΡΡΠ΅ ΠΏΡΠΈΠ²Π΅Π΄Π΅Π½Ρ Π΄Π°Π½Π½ΡΠ΅ ΠΎΠ± ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΈ, Π² ΠΊΠΎΡΠΎΡΠΎΠ΅ Π²ΠΎΡΠ»ΠΈ 76 Π΄Π΅ΡΠ΅ΠΉ ΠΏΠ΅ΡΠ²ΡΡ
2 Π»Π΅Ρ ΠΆΠΈΠ·Π½ΠΈ Ρ ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΠΌΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΠΌΠΈ Π»Π΅Π³ΠΊΠΈΡ
(ΠΠΠ). ΠΡΠΈ ΠΎΡΠ΅Π½ΠΊΠ΅ ΡΠΈΠΌΠΏΡΠΎΠΌΠΎΠ², Π²Ρ
ΠΎΠ΄ΡΡΠΈΡ
Π² Π΄Π΅ΡΡΠΊΠΈΠΉ ΠΠΠ-ΡΠΈΠ½Π΄ΡΠΎΠΌ, Ρ Π²ΡΠ΅Ρ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π½Π°Π±Π»ΡΠ΄Π°Π»ΠΈΡΡ ΠΎΠ±ΡΠ΅ΠΊΡΠΈΠ²Π½ΡΠ΅ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΡΠΈΠΌΠΏΡΠΎΠΌΡ ΠΈ ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΠ΅ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΡ ΠΏΠΎ Π΄Π°Π½Π½ΡΠΌ ΡΠ΅Π½ΡΠ³Π΅Π½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΉ. Π£ 93,4% ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΎΡΠΌΠ΅ΡΠ°Π»ΠΈΡΡ ΡΠ΅ΡΠΏΠΈΡΠ°ΡΠΎΡΠ½ΡΠ΅ ΡΠΈΠΌΠΏΡΠΎΠΌΡ, Ρ 68,4% ΠΏΠΎ Π΄Π°Π½Π½ΡΠΌ ΠΏΡΠ»ΡΡΠΎΠΊΡΠΈΠΌΠ΅ΡΡΠΈΠΈ Π±ΡΠ»Π° Π²ΡΡΠ²Π»Π΅Π½Π° Π³ΠΈΠΏΠΎΠΊΡΠ΅ΠΌΠΈΡ, ΡΡΠΎ ΠΏΠΎΠ·Π²ΠΎΠ»ΠΈΠ»ΠΎ ΡΡΡΠ°Π½ΠΎΠ²ΠΈΡΡ Π΄Π΅ΡΡΠΊΠΈΠΉ ΠΠΠ-ΡΠΈΠ½Π΄ΡΠΎΠΌ Ρ Π²ΡΠ΅Ρ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ², Π° Π½Π° ΠΎΡΠ½ΠΎΠ²Π°Π½ΠΈΠΈ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½Π½ΡΡ
ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΈ Π»Π°Π±ΠΎΡΠ°ΡΠΎΡΠ½ΠΎ-ΠΈΠ½ΡΡΡΡΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΡΡ
ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΉ Π±ΡΠ»Π° ΡΡΠΎΡΠ½Π΅Π½Π° Π½ΠΎΠ·ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠ°Ρ ΡΠΎΡΠΌΠ°. ΠΠΠ Ρ Π½Π°Π±Π»ΡΠ΄Π°Π²ΡΠΈΡ
ΡΡ Π΄Π΅ΡΠ΅ΠΉ Π²ΠΊΠ»ΡΡΠ°Π»ΠΈ Π²ΡΠΎΠΆΠ΄Π΅Π½Π½ΡΡ Π°Π»ΡΠ²Π΅ΠΎΠ»ΡΡΠ½ΠΎ-ΠΊΠ°ΠΏΠΈΠ»Π»ΡΡΠ½ΡΡ Π΄ΠΈΡΠΏΠ»Π°Π·ΠΈΡ (1,3%), Π»Π΅Π³ΠΎΡΠ½ΡΡ Π³ΠΈΠΏΠΎΠΏΠ»Π°Π·ΠΈΡ (17%), ΡΠΈΠ½Π΄ΡΠΎΠΌ ΠΠΈΠ»ΡΡΠΎΠ½Π°-ΠΠΈΠΊΠΈΡΠΈ (21,1%), ΡΡΠ±ΠΏΠ»Π΅Π²ΡΠ°Π»ΡΠ½ΡΠ΅ ΠΊΠΈΡΡΡ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠΈΠ½Π΄ΡΠΎΠΌΠΎΠΌ ΠΠ°ΡΠ½Π° (6,6%), Π½Π΅ΠΉΡΠΎΡΠ½Π΄ΠΎΠΊΡΠΈΠ½Π½ΡΡ Π³ΠΈΠΏΠ΅ΡΠΏΠ»Π°Π·ΠΈΡ ΠΌΠ»Π°Π΄Π΅Π½ΡΠ΅Π² (23,7%), Π²ΡΠΎΠΆΠ΄Π΅Π½Π½ΡΠΉ Π΄Π΅ΡΠΈΡΠΈΡ ΡΡΡΡΠ°ΠΊΡΠ°Π½ΡΠ½ΠΎΠ³ΠΎ ΠΏΡΠΎΡΠ΅ΠΈΠ½Π° Π (1,3%), ΡΠΈΠ½Π΄ΡΠΎΠΌ Β«ΠΌΠΎΠ·Π³-Π»Π΅Π³ΠΊΠΈΠ΅-ΡΠΈΡΠΎΠ²ΠΈΠ΄Π½Π°Ρ ΠΆΠ΅Π»Π΅Π·Π°Β» (2,6%), ΠΎΠ±Π»ΠΈΡΠ΅ΡΠΈΡΡΡΡΠΈΠΉ Π±ΡΠΎΠ½Ρ
ΠΈΠΎΠ»ΠΈΡ Ρ ΠΎΡΠ³Π°Π½ΠΈΠ·ΡΡΡΠ΅ΠΉΡΡ ΠΏΠ½Π΅Π²ΠΌΠΎΠ½ΠΈΠ΅ΠΉ (10,5%), ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΠ΅ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΡ Π»Π΅Π³ΠΊΠΈΡ
ΠΏΡΠΈ ΡΠΈΡΡΠ΅ΠΌΠ½ΡΡ
Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΡ
(Π³ΠΈΡΡΠΈΠΎΡΠΈΡΠΎΠ· ΠΈΠ· ΠΊΠ»Π΅ΡΠΎΠΊ ΠΠ°Π½Π³Π΅ΡΠ³Π°Π½ΡΠ° - 14,6%, Π±ΠΎΠ»Π΅Π·Π½Ρ ΠΠΈΠΌΠ°Π½Π½Π°-ΠΠΈΠΊΠ° - 1,3%). ΠΡΠΎΠ°Π½Π°Π»ΠΈΠ·ΠΈΡΠΎΠ²Π°Π½Ρ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΉ ΠΊΠ°ΡΡΠΈΠ½Ρ, Π΄Π°Π½Π½ΡΠ΅ Π»Π°Π±ΠΎΡΠ°ΡΠΎΡΠ½ΠΎΠΈΠ½ΡΡΡΡΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΡΡ
ΠΌΠ΅ΡΠΎΠ΄ΠΎΠ² ΠΎΠ±ΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ, ΡΡΠΆΠ΅ΡΡΡ ΡΠ΅ΡΠ΅Π½ΠΈΡ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ, ΠΏΡΠΎΠ³Π½ΠΎΠ·, Π²ΡΡΠ²Π»Π΅Π½Ρ ΠΏΡΠ΅Π΄ΠΈΠΊΡΠΎΡΡ Π»Π΅ΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΈΡΡ
ΠΎΠ΄Π° Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ Π΄Π°Π½Π½ΡΠΌΠΈ ΡΠ΅Π΄ΠΊΠΈΠΌΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΠΌΠΈ, Π² ΡΠΎΠΌ ΡΠΈΡΠ»Π΅ Ρ Π½Π΅Π΄ΠΎΠ½ΠΎΡΠ΅Π½Π½ΡΡ
Π΄Π΅ΡΠ΅ΠΉ
ΠΠΎΠ·ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠ°Ρ ΡΡΡΡΠΊΡΡΡΠ° ΠΈ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΡ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ Π»Π΅Π³ΠΊΠΈΡ Ρ Π΄Π΅ΡΠ΅ΠΉ ΠΏΠ΅ΡΠ²ΡΡ 2 Π»Π΅Ρ ΠΆΠΈΠ·Π½ΠΈ: ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΡ ΠΌΠ½ΠΎΠ³ΠΎΡΠ΅Π½ΡΡΠΎΠ²ΠΎΠ³ΠΎ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ
The article presents data about study included 76 children of the first two years of life with interstitial lung diseases (ILD). According to symptoms of chILD-syndrome, all patients had respiratory signs and interstitial changes on X-ray. 93.4% of patients had respiratory symptoms, hypoxemia was revealed in 68.4% of patients by pulsoximetry. The presence of those symptoms allowed to establish chILD-syndrome in all patients, and, on the basis of clinical and laboratory-instrumental studies, nosological form was refined. Children of our study have forms of IlD such as congenital alveolar-capillary dysplasia (1.3%), pulmonary hypoplasia (17%), Wilson-Mikity syndrome (21.1%), subpleural cysts in patients with Down syndrome (6.6%), neuroendocrine cell hyperplasia of infancy (23.7%), congenital deficiency of surfactant protein B (1.3%), brain-lung-thyroid syndrome (2.6%), bronchiolitis obliterans with organizing pneumonia (10.5%), disorders related to systemic disease processes (Langerhans cell histiocytosis - 14.6%, Niemann-Pick disease - 1.3%). The features of clinical picture, data of laboratory-instrumental methods of investigation, severity of the disease's course, prognosis, predictors of the death outcome in patients with these rare diseases, have been analyzed in all patients including preterm infants.Π ΡΡΠ°ΡΡΠ΅ ΠΏΡΠΈΠ²Π΅Π΄Π΅Π½Ρ Π΄Π°Π½Π½ΡΠ΅ ΠΎΠ± ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΈ, Π² ΠΊΠΎΡΠΎΡΠΎΠ΅ Π²ΠΎΡΠ»ΠΈ 76 Π΄Π΅ΡΠ΅ΠΉ ΠΏΠ΅ΡΠ²ΡΡ
2 Π»Π΅Ρ ΠΆΠΈΠ·Π½ΠΈ Ρ ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΠΌΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΠΌΠΈ Π»Π΅Π³ΠΊΠΈΡ
(ΠΠΠ). ΠΡΠΈ ΠΎΡΠ΅Π½ΠΊΠ΅ ΡΠΈΠΌΠΏΡΠΎΠΌΠΎΠ², Π²Ρ
ΠΎΠ΄ΡΡΠΈΡ
Π² Π΄Π΅ΡΡΠΊΠΈΠΉ ΠΠΠ-ΡΠΈΠ½Π΄ΡΠΎΠΌ, Ρ Π²ΡΠ΅Ρ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π½Π°Π±Π»ΡΠ΄Π°Π»ΠΈΡΡ ΠΎΠ±ΡΠ΅ΠΊΡΠΈΠ²Π½ΡΠ΅ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠ΅ ΡΠΈΠΌΠΏΡΠΎΠΌΡ ΠΈ ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΠ΅ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΡ ΠΏΠΎ Π΄Π°Π½Π½ΡΠΌ ΡΠ΅Π½ΡΠ³Π΅Π½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΉ. Π£ 93,4% ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΎΡΠΌΠ΅ΡΠ°Π»ΠΈΡΡ ΡΠ΅ΡΠΏΠΈΡΠ°ΡΠΎΡΠ½ΡΠ΅ ΡΠΈΠΌΠΏΡΠΎΠΌΡ, Ρ 68,4% ΠΏΠΎ Π΄Π°Π½Π½ΡΠΌ ΠΏΡΠ»ΡΡΠΎΠΊΡΠΈΠΌΠ΅ΡΡΠΈΠΈ Π±ΡΠ»Π° Π²ΡΡΠ²Π»Π΅Π½Π° Π³ΠΈΠΏΠΎΠΊΡΠ΅ΠΌΠΈΡ, ΡΡΠΎ ΠΏΠΎΠ·Π²ΠΎΠ»ΠΈΠ»ΠΎ ΡΡΡΠ°Π½ΠΎΠ²ΠΈΡΡ Π΄Π΅ΡΡΠΊΠΈΠΉ ΠΠΠ-ΡΠΈΠ½Π΄ΡΠΎΠΌ Ρ Π²ΡΠ΅Ρ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ², Π° Π½Π° ΠΎΡΠ½ΠΎΠ²Π°Π½ΠΈΠΈ ΠΏΡΠΎΠ²Π΅Π΄Π΅Π½Π½ΡΡ
ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΈ Π»Π°Π±ΠΎΡΠ°ΡΠΎΡΠ½ΠΎ-ΠΈΠ½ΡΡΡΡΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΡΡ
ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΉ Π±ΡΠ»Π° ΡΡΠΎΡΠ½Π΅Π½Π° Π½ΠΎΠ·ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠ°Ρ ΡΠΎΡΠΌΠ°. ΠΠΠ Ρ Π½Π°Π±Π»ΡΠ΄Π°Π²ΡΠΈΡ
ΡΡ Π΄Π΅ΡΠ΅ΠΉ Π²ΠΊΠ»ΡΡΠ°Π»ΠΈ Π²ΡΠΎΠΆΠ΄Π΅Π½Π½ΡΡ Π°Π»ΡΠ²Π΅ΠΎΠ»ΡΡΠ½ΠΎ-ΠΊΠ°ΠΏΠΈΠ»Π»ΡΡΠ½ΡΡ Π΄ΠΈΡΠΏΠ»Π°Π·ΠΈΡ (1,3%), Π»Π΅Π³ΠΎΡΠ½ΡΡ Π³ΠΈΠΏΠΎΠΏΠ»Π°Π·ΠΈΡ (17%), ΡΠΈΠ½Π΄ΡΠΎΠΌ ΠΠΈΠ»ΡΡΠΎΠ½Π°-ΠΠΈΠΊΠΈΡΠΈ (21,1%), ΡΡΠ±ΠΏΠ»Π΅Π²ΡΠ°Π»ΡΠ½ΡΠ΅ ΠΊΠΈΡΡΡ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠΈΠ½Π΄ΡΠΎΠΌΠΎΠΌ ΠΠ°ΡΠ½Π° (6,6%), Π½Π΅ΠΉΡΠΎΡΠ½Π΄ΠΎΠΊΡΠΈΠ½Π½ΡΡ Π³ΠΈΠΏΠ΅ΡΠΏΠ»Π°Π·ΠΈΡ ΠΌΠ»Π°Π΄Π΅Π½ΡΠ΅Π² (23,7%), Π²ΡΠΎΠΆΠ΄Π΅Π½Π½ΡΠΉ Π΄Π΅ΡΠΈΡΠΈΡ ΡΡΡΡΠ°ΠΊΡΠ°Π½ΡΠ½ΠΎΠ³ΠΎ ΠΏΡΠΎΡΠ΅ΠΈΠ½Π° Π (1,3%), ΡΠΈΠ½Π΄ΡΠΎΠΌ Β«ΠΌΠΎΠ·Π³-Π»Π΅Π³ΠΊΠΈΠ΅-ΡΠΈΡΠΎΠ²ΠΈΠ΄Π½Π°Ρ ΠΆΠ΅Π»Π΅Π·Π°Β» (2,6%), ΠΎΠ±Π»ΠΈΡΠ΅ΡΠΈΡΡΡΡΠΈΠΉ Π±ΡΠΎΠ½Ρ
ΠΈΠΎΠ»ΠΈΡ Ρ ΠΎΡΠ³Π°Π½ΠΈΠ·ΡΡΡΠ΅ΠΉΡΡ ΠΏΠ½Π΅Π²ΠΌΠΎΠ½ΠΈΠ΅ΠΉ (10,5%), ΠΈΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΡΠ΅ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΡ Π»Π΅Π³ΠΊΠΈΡ
ΠΏΡΠΈ ΡΠΈΡΡΠ΅ΠΌΠ½ΡΡ
Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΡ
(Π³ΠΈΡΡΠΈΠΎΡΠΈΡΠΎΠ· ΠΈΠ· ΠΊΠ»Π΅ΡΠΎΠΊ ΠΠ°Π½Π³Π΅ΡΠ³Π°Π½ΡΠ° - 14,6%, Π±ΠΎΠ»Π΅Π·Π½Ρ ΠΠΈΠΌΠ°Π½Π½Π°-ΠΠΈΠΊΠ° - 1,3%). ΠΡΠΎΠ°Π½Π°Π»ΠΈΠ·ΠΈΡΠΎΠ²Π°Π½Ρ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΉ ΠΊΠ°ΡΡΠΈΠ½Ρ, Π΄Π°Π½Π½ΡΠ΅ Π»Π°Π±ΠΎΡΠ°ΡΠΎΡΠ½ΠΎΠΈΠ½ΡΡΡΡΠΌΠ΅Π½ΡΠ°Π»ΡΠ½ΡΡ
ΠΌΠ΅ΡΠΎΠ΄ΠΎΠ² ΠΎΠ±ΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ, ΡΡΠΆΠ΅ΡΡΡ ΡΠ΅ΡΠ΅Π½ΠΈΡ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ, ΠΏΡΠΎΠ³Π½ΠΎΠ·, Π²ΡΡΠ²Π»Π΅Π½Ρ ΠΏΡΠ΅Π΄ΠΈΠΊΡΠΎΡΡ Π»Π΅ΡΠ°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΈΡΡ
ΠΎΠ΄Π° Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ Π΄Π°Π½Π½ΡΠΌΠΈ ΡΠ΅Π΄ΠΊΠΈΠΌΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΠΌΠΈ, Π² ΡΠΎΠΌ ΡΠΈΡΠ»Π΅ Ρ Π½Π΅Π΄ΠΎΠ½ΠΎΡΠ΅Π½Π½ΡΡ
Π΄Π΅ΡΠ΅ΠΉ
The structure of interstitial lung diseases in children of the first two years of life
For the first time in Russia, the article provides data on interstitial lung diseases structure in children of the first two years of life, based on a series of observations of 68 patients with these rare diseases, as a part of multi-center ambispective study. Interstitial lung diseases in observed children included: Wilson-Mikity syndrome (23,4%), neuroendocrine hyperplasia of infancy (22%), bronchiolitis obliterans with organizing pneumonia (7,4%), primary pulmonary hypoplasia (1,5%), secondary pulmonary hypoplasia with Jeune syndrome (10,3%), secondary pulmonary hypoplasia with Edwards syndrome (2,9%), secondary pulmonary hypoplasia with other associated pathology (omphalocele - 1,5%, non-immune fetal hydrops - 1,5%), subpleural cysts in patients with Down syndrome (5,9%), congenital deficiency of surfactant protein B (1,5%), brain-lung- thyroid syndrome (2,9%), congenital alveolar-capillary dysplasia (1,5%), interstitial lung diseases with systemic diseases (Langerhans cell histiocytosis - 16,2%, Niemann-Pick disease - 1,5%). The article summarizes clinical features, the results of image diagnosis and disease outcomes. Β© 2015, Pediatria Ltd. All rights reserved
The structure of interstitial lung diseases in children of the first two years of life
For the first time in Russia, the article provides data on interstitial lung diseases structure in children of the first two years of life, based on a series of observations of 68 patients with these rare diseases, as a part of multi-center ambispective study. Interstitial lung diseases in observed children included: Wilson-Mikity syndrome (23,4%), neuroendocrine hyperplasia of infancy (22%), bronchiolitis obliterans with organizing pneumonia (7,4%), primary pulmonary hypoplasia (1,5%), secondary pulmonary hypoplasia with Jeune syndrome (10,3%), secondary pulmonary hypoplasia with Edwards syndrome (2,9%), secondary pulmonary hypoplasia with other associated pathology (omphalocele - 1,5%, non-immune fetal hydrops - 1,5%), subpleural cysts in patients with Down syndrome (5,9%), congenital deficiency of surfactant protein B (1,5%), brain-lung- thyroid syndrome (2,9%), congenital alveolar-capillary dysplasia (1,5%), interstitial lung diseases with systemic diseases (Langerhans cell histiocytosis - 16,2%, Niemann-Pick disease - 1,5%). The article summarizes clinical features, the results of image diagnosis and disease outcomes. Β© 2015, Pediatria Ltd. All rights reserved